Secrets – Pediatric: Dermatology
ACNE
1. When is acne most likely to develop?
The development of micro comedones is typically the earliest sign of acne. Studies have shown that comedones occur in three fourths of premenarchal girls at an average age of 10 years and in about half of 10- to 11-year-old boys. They may herald (or predate) the onset of puberty.
2. When are pimples precocious?
Acne vulgaris that begins before age 7 years should warrant further investigation for endocrine abnormalities such as androgen excess or precocious puberty.
Eichenfield LF, Krakowski AC, Piggott C, et al: Evidence-based recommendations for the diagnosis and treatment of pediatric acne, Pediatrics 131:S163–S185, 2013.
3. Which skin structure is involved in acne pathogenesis?
The pilosebaceous unit. It consists of a vellus hair follicle in association with sebaceous glands, which secrete sebum into the lumen of the follicle.
4. What are the four key factors in acne pathogenesis?
1. Androgen–dependent sebum production
2. Abnormal follicular keratinization, which leads to follicular plugging
3. Proliferation of Propionibacterium acnes bacteria that live in the lumen of the follicle and thrive in an anaerobic environment; P. acnes is lipolytic and breaks down sebum, releasing mediators of inflammation
4. Inflammation
5. Are blackheads caused by dirty skin?
No. The black color of a “blackhead,” or open comedone, is caused by the mass of sebum and compact keratin debris that has oxidized at the follicular opening. Whiteheads, also called closed comedones, occur when the contents tent the overlying skin but are not exposed to the atmosphere.
6. What is the difference between neonatal acne and infantile acne? Neonatal or “baby” acne occurs in up to 20% of newborns and typically presents during the first 4 weeks after birth. Erythematous papulopustules develop on the face, especially the cheeks. It has been attributed to the transient elevation of androgenic hormones (both maternally derived and endogenous) that are present in a newborn infant. The lesions typically resolve within 1 to 3 months as androgen levels fall. Neonatal cephalic pustulosis is a term that has been proposed to replace neonatal acne. Because lesions have been shown to contain Malassezia species, neonatal “acne” may actually represent an inflammatory reaction to this yeast flora and not true acne at all.
Infantile acne is uncommon and usually presents on a delayed basis (3 to 6 months). Histologically, the lesions are similar to true acne vulgaris as seen in older children: open and closed comedones; inflammatory papules; and rarely, nodules may be present. An examination for signs of androgen excess is indicated although most patients with this condition have no evidence of precocious puberty or increased hormonal levels. Systemic therapy may be required to minimize scarring.
aDisclaimer: Although off-label use of medications is discussed in this chapter, it is not designed to provide specific treatment
guidelines.
bConflict of interest: Dr. Kimberly Morel: Galderma, Scientific Advisory Board Meeting, July 2013. Pierre Fabre, Scientific Advisory Board Meeting, September 2013. Drs. Kimberly Morel, Christine Lauren and Maria Garzon, Astellas Pharma, Inc., grant support to University.
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KEY POINTS: MORPHOLOGIC DESCRIPTIONS OF PRIMARY CUTANEOUS LESIONS
1. Macule: A circumscribed, flat area, recognizable by color variation from surrounding skin, ≤1 cm
2. Patch: A large macule, >1 cm
3. Papule: A circumscribed elevation, ≤1 cm
4. Plaque: A large superficial papule, >1 cm
5. Nodule: A circumscribed solid elevation, ≤1 cm
6. Vesicle (small blister): A clear, fluid-filled elevation, ≤1 cm
7. Bulla (large blister): A fluid-filled elevation, >1 cm
8. Pustule: A circumscribed elevation of skin filled with pus
7. Which disorders resemble neonatal and infantile acne?
• Miliaria rubra or pustulosa, often in areas of occlusion and skin folds
• Milia, white papules without surrounding erythema
• Sebaceous hyperplasia, yellowish papules typically on the nose
• Seborrheic dermatitis, erythematous scaly patches rather than pustules.
8. Is an infant with acne more likely to be a teenager with acne?
The presence or severity of acne in an infant who is <3 months old is not believed to correlate with an increased likelihood of adolescent acne. However, delayed acne between 3 and 6 months of age (especially if persistent and severe) is associated with a higher likelihood of more
severe adolescent disease. Family history of severe acne also increases the likelihood of future problems.
Herane MI, Ando I: Acne in infancy and acne genetics, Dermatology 206:24–28, 2003.
9. Which factors exacerbate acne?
• Vigorous scrubbing or picking of lesions
• Use of comedogenic makeup or other facial products
• Medications: anabolic steroids and corticosteroids, lithium, barbiturates, and some oral contraceptives
• Sweating and tight-fitting clothes or sports equipment
• Hormone dysregulation, such as polycystic ovarian syndrome
• Studies are evaluating the influence of high glycemic index diets and insulin resistance
KEY POINTS: MAIN FACTORS IN ACNE PATHOGENESIS
1. Androgen–dependent sebum production
2. Abnormal follicular keratinization
3. Proliferation of Propionobacterium acnes
4. Inflammation
10. What are the most severe forms of acne?
Acne fulminans is a rare but severe disorder that has also been called acute febrile ulcerative acne. It occurs mainly in teenage boys as extensive, inflammatory, ulcerating lesions on the trunk and chest that are usually associated with fever, malaise, arthralgia, and leukocytosis. The etiology remains unclear, but immune complexes are thought to be involved. Treatment is systemic and includes the following: antibiotics, glucocorticoids, and retinoids.
Acne conglobata is a severe form of acne that presents with comedones, papules, pustules, nodules, and abscesses. It is associated with significant scarring. It often arises in early adulthood, more typically in females. Systemic retinoid therapy is the treatment of choice.
James WD: Acne, N Engl J Med 352:1463–1472, 2005.
11. What is the therapeutic approach to acne?
Acne therapies, including comedolytics, antibacterial agents, and hormonal modulators, target various factors involved in the pathogenesis of acne.
• Topical antibiotics including erythromycin and clindamycin should be used in combination with benzoyl peroxide (BPO) to decrease the risk of Propionibacterium acnes antibiotic resistance. BPO itself is bactericidal against P. acnes.
• Systemic antibiotics (e.g., tetracycline and its derivatives) are most frequently used for moderate-to-severe papulopustular acne.
• Topical retinoids tretinoin, tazarotene (pregnancy category X), and adapalene are comedolytic agents that prevent the formation of new keratin plugs.
• Systemic retinoids (isotretinoin) are used in cases of severe acne vulgaris. The exact mechanism of action of isotretinoin is not known but affects all four factors in acne pathogenesis: keratinization/ follicular plugging, inhibition of sebaceous gland activity, reduced P. acnes, and inflammation.
• Hormonal modulation is most commonly accomplished with oral contraceptives. Select oral contraceptives are Food and Drug Administration (FDA) approved to treat moderate to severe acne in menstruating females over age 14 years. Antiandrogenic agents such as spironolactone
have been used in some teenage females with premenstrual flares, hirsutism, and male-pattern alopecia.
Eichenfield LF, Krakowski AC, Piggott C, et al: Evidence-based recommendations for the diagnosis and treatment of pediatric acne, Pediatrics 131:S163–S185, 2013.
12. When is the use of oral isotretinoin indicated in teenagers with acne?
Isotretinoin, which is 13-cis-retinoic acid, is most appropriately used for nodulocystic acne, acne conglobata, or scarring acne that has been unresponsive to standard modes of treatment
(e.g., oral/topical antibiotics, topical retinoids). Given the known side effect of teratogenicity if even one dose of isotretinoin is taken during pregnancy or if a female becomes pregnant within 30 days of the last dose, rigorous monitoring and definitive contraceptive counseling are mandatory. The FDA and the manufacturers of isotretinoin created a registry, called iPLEDGE (www.ipledgeprogram.com), in an effort to reduce the risk of fetal exposure to isotretinoin. This program monitors patients on isotretinoin with monthly lab tests and verification of contraception and knowledge of risks.
Eichenfield LF, Krakowski AC, Piggott C, et al: Evidence-based recommendations for the diagnosis and treatment of pediatric acne, Pediatrics 131:S163–185, 2013.
Merritt B, Burkhart CN, Morrell DS: Use of isotretinoin for acne vulgaris, Pediatr Ann 38:311–320, 2009.
13. What serious side effects may be associated with systemic minocycline therapy for acne?
Tetracyclines, including the derivative minocycline, are widely prescribed oral antibiotics for acne and have been used safely over long periods of time. They are contraindicated for patients <8 years old because of the potential for permanent dental staining. Rare reactions—particularly to minocycline—have included skin discoloration, pneumonitis, autoimmune hepatitis, drug-induced lupus, serum-sickness-like reactions, and severe hypersensitivity reactions.
Brown RJ, Rother KI, Artman H, et al: Minocycline-induced drug hypersensitivity syndrome followed by multiple autoimmune sequelae, Arch Dermatol 142:862–868, 2009.
14. Which combination of acne products will cause a yellow-orange skin and hair discoloration?
The use of topical dapsone 5% gel (Aczone a off-label under age 12 years) plus BPO will lead to a yellow- orange skin discoloration. Patients should be warned to avoid applying these medications at the same time. Topical sulfacetamide applied at the same time as BPO will also cause this reaction. If alternating application morning and night, a gentle cleansing should occur between each step. Once the reaction occurs, it may take from a few days up to 2 months to resolve.
Dubina MI, Fleischer AB Jr; Interaction of topical sulfacetamide and topical dapsone with benzoyl peroxide, Arch Dermatol 145:1027–1029, 2009.
15. What other topical acne products should not be used in combination?
The combination of topical tretinoin plus BPO applied at the same time is known to cause oxidation and inactivity of the tretinoin. Tretinoin is also inactivated by sunlight, so it is recommended to apply it in the evening. BPO may be used by the patient the next morning. A topical combination product (Epiduob) has been developed that contains adapalene in a stable combination with 2.5% BPO. It is FDA approved to treat acne in patients who are ≤9 years of age.
16. What color will your red towel become after you wipe your BPO-covered face with it? Pink or even white! The oxidizing effect of BPO has a bactericidal effect on P. acnes; the downside is the oxidizing effect will bleach or fade clothing, towels, and bedsheets. Patients should be warned of this effect and to use caution when applying clothing after recent application of BPO.
CLINICAL ISSUES
17. What skin findings in the midline lumbosacral region are suggestive of occult spinal dysraphism?
• Lipoma
• Hypertrichosis
• Pits: Sinuses or large dermal dimples (>0.5 cm) that are located >2.5 cm from the anal verge (particularly with lateral deviation of the cleft)
• Vascular lesions (hemangioma (Fig. 4-1), port wine stain, telangiectasias)
• Pigmentation variants (both hyperpigmentation, including lentigo and melanocytic nevus, and hypopigmentation)
• Aplasia cutis congenita
• Appendages (skin tags, tail)
Drolet BA, Chamlin SL, Garzon MC, et al: Prospective study of spinal anomalies in children with infantile hemangiomas of the lumbosacral skin, J Pediatr 157:789–794, 2010.
Drolet B: Birthmarks to worry about. Cutaneous markers of dysraphism, Dermatol Clin 16:447–453, 1998.
Figure 4-1. Lumbosacral hemangioma with underlying tethered cord. (From Drolet BA, Garzon MC, editors: Birthmarks of medical significance, Pediatr Clin North Am 57:1077, 2010.)
KEY POINTS: MIDLINE LUMBOSACRAL LESIONS ASSOCIATED WITH OCCULT SPINAL DYSRAPHISM OR TETHERED CORD
1. Sacral pits (particularly with lateral deviation of the gluteal cleft)
2. Hairy patches
3. Appendages (skin tag or tail)
4. Sacral lipoma
5. Vascular lesions (hemangioma, port wine stain, telangiectasias)
6. Pigmentation variants (hyperpigmentation, including lentigo and melanocytic nevus, and hypopigmentation)
7. Aplasia cutis congenita
18. What is the significance of accessory tragi?
Accessory tragi are fleshy papules or nodules that are typically anterior to the normal tragus, or less commonly on the cheek or jawline. They contain variable amounts of cartilage. In most cases, accessory tragi are isolated cutaneous findings. Extensive defects may be associated with hearing loss. Newborns with accessory tragi should have their hearing tested. Less commonly, they are associated with other first branchial arch abnormalities (e.g., cleft lip and palate) or rare syndromes such as Treacher Collins, VACTERL or oculoauriculovertebral syndrome. Accessory tragi are treated by surgically excising the papule and its cartilaginous stalk.
19. What conditions cause ringlike rashes on the skin? Not all rings are ringworm. Annular (ringlike) skin lesions can be seen in a wide variety of skin diseases in children. Common causes of these lesions include the following:
• Tinea corporis
• Dermatitis (especially nummular)
• Psoriasis
• Urticaria
• Granuloma annulare (often composed of small papules without overlying scale)
• Erythema migrans
• Systemic lupus erythematosus
KEY POINTS: DIFFERENTIAL DIAGNOSES OF RINGLIKE SKIN RASHES
1. Tinea corporis
2. Dermatitis (atopic, nummular, or contact)
3. Psoriasis
4. Granuloma annulare (often composed of small papules without overlying scale)
5. Erythema migrans
6. Systemic lupus erythematosus
20. What is the appearance and natural history of molluscum contagiosum? Molluscum contagiosum is a common skin infection caused by a poxvirus. Lesions are small pinkish-tan, dome-shaped papules that often have a dimpled or umbilicated center. They are usually asymptomatic, but they may be associated with an eczematous dermatitis and itch. Superinfection may complicate the course, require antibiotic therapy, and increase the likelihood of scarring after resolution. In healthy children, the course is self-limited but may last for 2 years. In some cases, persistent and widespread molluscum may require screening for congenital or acquired immunodeficiencies.
21. What is the best way to eradicate molluscum contagiosum?
If watchful waiting is not desired, therapeutic options are primarily destructive methods. Curettage (with core removal), cryotherapy, and peeling agents (topical retinoids applied sparingly) can be used. A popular method involves the use of the blistering agent cantharidin,a which is applied to individual lesions in the physician’s office. Immunomodulating (e.g., imiquimod,a cimetidinea) and antiviral therapies remain unproved in children.
Moye VA, Cathcart S, Morrell DS: Safety of cantharidin: a retrospective review of cantharidin treatment in 405 children with molluscum contagiosum, Pediatr Dermatol 31:450–454, 2014.
22. What are the common causes of acute urticaria in children? Acute urticaria may last for several weeks. If it persists beyond that period, it is typically characterized as chronic urticaria. In children, the most common causes of acute urticaria include the five “I’s”:
• Infection (viral and bacterial are the most frequent, but fungal pathogens may also cause urticaria)
• Infestation (parasites)
• Ingestion (medication and foods)
• Injections or infusions (immunizations, blood products, and antibiotics)
• Inhalation (allergens such as pollens and molds)
Weston W, Orchard D: Vascular reactions. In Schachner LA, Hansen RC, editors: Pediatric Dermatology, ed 3. St. Louis, 2003, Mosby, pp 801–831.
KEY POINTS: COMMON CAUSES OF URTICARIA— THE FIVE I’ S
1. Infection (viral and bacterial are the most frequent, but fungal pathogens may also cause urticaria)
2. Infestation (parasites)
3. Ingestion (medications and foods)
4. Injections or infusions (immunizations, blood products, and antibiotics)
5. Inhalation (allergens such as pollens and molds)
23. What is the characteristic clinical picture of erythema nodosum? A prodrome of fever, chills, malaise, and arthralgia may precede the typical skin findings. Crops of red to blue tender nodules appear over the anterior shins. Lesions may be seen on the knees, ankles, thighs, and, occasionally, the lower extensor forearms and face. They may evolve through a spectrum of colors that resemble a bruise. Often the changes are misdiagnosed as cellulitis or secondary to a traumatic event. This condition is associated with a variety of infectious (e.g., group A beta hemolytic streptococcus, tuberculosis) and noninfectious (e.g., ulcerative colitis, leukemia) causes.
24. What, technically, are warts?
Benign epidermal tumors caused by multiple types of human papillomaviruses.
25. How are plantar warts distinguished clinically from calluses? Plantar warts are warts on the soles of the feet that can be painful. They are flat or slightly raised areas of firm hyperkeratosis with a collarette of normal skin (Fig. 4-2). Unlike calluses, with which they can be confused, plantar warts cause obliteration of the normal skin lines (dermatoglyphics). Pinpoint-sized dark red dots (thrombosed capillaries) may be seen within the wart.
Figure 4-2. Plantar warts. Note disruption of skin lines. Characteristic black dots in the warts are thrombosed capillaries. (From Cohen BA: Pediatric Dermatology, ed 2. London, 1999,
Mosby, p 115.)
26. How can common warts be treated? The mode of therapy depends on the type and number of warts, the location on the body, and the age of the patient. No matter what treatment is used, warts can always recur; there are no absolute cures. The major goal is to remove warts without residual scarring. Of course, another option is no treatment at all because most warts self-resolve, but they may take years to do so. A variety of therapies are used. The most commonly used therapy is salicylic acid with regular paring and occlusion. The second is cryotherapy (topical liquid nitrogen) in combination with salicylic acid. Other therapies including topical tretinoin, duct tape application, electrodessication, pulsed dye laser, topical imiquimod, and contact immunotherapy have been used to treat recalcitrant warts in children.a In some case reports, oral cimetidine has been
reported to be effective, perhaps because of its immunomodulatory activity, although an evidence base is lacking.a Candida antigen injection as an immunotherapy has also been reported to be efficacious.a
Lynch MD, Cliffe J, Morris-Jones R: Management of cutaneous viral warts, BMJ 348:g3339, 2014.
Swanson A, Canty K: Common pediatric skin conditions with protracted courses: a therapeutic update, Dermatol Clin
31:239–249, 2013.
27. An 8-year-old has a hard, nontender, freely mobile nodule of the neck with a slightly bluish hue of the skin. What is the most likely diagnosis? Pilomatricoma (pilomatrixoma). Also called the benign calcifying epithelioma of Malherbe, it is a benign tumor that often arises in children and adolescents on the face and neck. It is usually not confused with a malignant condition, but excision is often recommended because these nodules increase in size or may become inflamed or infected.
28. What is the “teeter-totter sign”? A clue to the diagnosis of pilomatricomas. Like children playing on a see-saw, when one side goes up, the other goes down. Pilomatricomas are firm to rock hard, and when one end is pressed down, the other lifts.
29. What are the most common causes of lumps and bumps in the skin of children? Although most parents fear malignancy, nodules and tumors in the skin are rarely malignant. Epidermal inclusion cysts are one of the most common causes and are often recognized by their central punctum. Pilomatricomas are another common pediatric cause (see questions 27 and 28). Even though benign, they should be removed because they may continue to enlarge over time and may become inflamed or infected. Deep infantile hemangiomas may develop in early infancy; they are soft and partially compressible but may be difficult to recognize if they not associated with the superficial red component of the hemangioma. Imaging may be indicated if there are no external cutaneous clues. Any rapidly progressive or firm, tender lumps should be evaluated promptly because a pediatric surgery referral may be required to assist with a definitive diagnosis and to rule out the rare cases of malignancy.
Wyatt AJ, Hansen RC: Pediatric skin tumors, Pediatr Clin North Am 47:937–963, 2000.
30. Why is a pyogenic granuloma neither pyogenic nor a granuloma? A pyogenic granuloma, which is also called a lobular capillary hemangioma, is a common acquired lesion that develops typically at the site of obvious or trivial trauma on any part of the body. Local capillary proliferation occurs, often rapidly, and bleeding may develop (Fig. 4-3). Curettage and electrodessication of the base are curative. The lesion is neither an infectious pyoderma nor a granuloma on biopsy.
Figure 4-3. Pyogenic granuloma in the web space between fingers. (From Cohen BA: Pediatric Dermatology, ed 2. London, 1999,
Mosby, p 127.)
31. What condition is classically diagnosed by the Darier sign? Mastocytoma. This is a benign lesion composed of mast cells that arises at birth or during early infancy. It appears as a pink/tan plaque or nodule, often with a peau d’orange surface. Darier sign refers to the eliciting of erythema and an urticarial wheal by stroking or rubbing the lesion. The skin changes are caused by the release of histamine from the mechanically traumatized mast cells.
32. What disorder can present as “freckles” associated with hives?
Urticaria pigmentosa (mastocytosis). Presenting at birth or during early infancy, multiple mastocytomas appear as brown macules, papules, or plaques (vesicle formation can also occur) and are often mistaken for freckles or melanocytic nevi. Lesions are usually only cutaneous but infrequently they may affect other organ systems (e.g., lungs, kidney, gastrointestinal tract, central nervous system). The Darier sign is a key feature of diagnosis.
33. What is impetigo? Impetigo is a superficial skin infection that is caused by Staphylococcus aureus or group A streptococcus. Historically, streptococcus was the most prevalent agent. However, over the last few decades, S. aureus appears to be the predominant organism, although mixed infections may also occur. Bullous
impetigo is usually caused by S. aureus.
34. Is topical or systemic therapy better for impetigo? Treatment usually requires an antibiotic that is active against both streptococci and staphylococci. Topical antibiotics can be used in localized disease. The components of over-the-counter triple antibiotic ointment (usually bacitracin-neomycin-polymyxin B) do have some activity against the pathogenic bacteria of impetigo, but mupirocin is more effective as a topical agent although resistance to mupirocin is on the rise. Systemic antibiotics, with activity against both S. aureus and group A streptococcus, are usually indicated for extensive involvement, outbreaks among household contacts, schools, or athletic teams, or if topical therapy has failed. Cephalosporins (e.g., cephalexin, cefadroxil), amoxicillin–clavulanate, and dicloxacillin are most effective. Erythromycin is unlikely to be useful because increasing numbers of staphylococci are resistant; local resistance patterns should determine if erythromycin can be used. Methicillin-resistant S. aureus (MRSA) is cultured as the causative agent of infections with more frequency. Most MRSA strains remain sensitive to trimethoprim-sulfamethoxazole and clindamycin.
Jungk J, Como-Sabetti K, Stinchfield P, et al: Epidemiology of MRSA at a pediatric healthcare system, Pediatr Infect Disease J 26:339–344, 2007.
Sladden MJ, Johnston GA: Common skin infections in children, BMJ 329:95–99, 2004.
35. What dermatologic sign starts from a scratch?
Dermographism (dermatographism) occurs when susceptible skin is stroked firmly with a pointed object. The result is a red line that is followed by an erythematous flare, which is eventually followed by a wheal (Fig. 4-4). This “triple response of Lewis” usually occurs within 1 to 3 minutes. Dermographism
Figure 4-4. Dermographism. An urticarial response is elicited by firm stroking of the skin. (From Goldbloom RB: Pediatric Clinical Skills, ed 4. Philadelphia, 2011, ELSEVIER Saunders.)
(or skin writing) is an exaggerated triple response of Lewis and is seen in patients with urticaria. The tendency to be dermographic can appear at any age and may last for months to years. The cause is often unknown. White dermographism is seen in patients with an atopic diathesis, in whom the red line is replaced by a white line without a subsequent flare and wheal.
36. Does a geographic tongue occur as a result of global travel?
No, so there is no need to stay home. Geographic tongue refers to the benign condition in which denudations of the filiform papillae on the lingual surface occur, giving the tongue the appearance of a relief map (Fig. 4-5). The patterns may change over hours and days, and the histopathology resembles that of psoriasis. The patient is usually asymptomatic. No treatment is effective or necessary
because self-resolution is the rule.
Figure 4-5. Geographic tongue. (From Sahn EE: Dermatology Pearls. Philadelphia, 1999, Hanley & Belfus, p 162.)
37. What diseases are associated with a strawberry tongue?
Scarlet fever caused by group A beta hemolytic streptococcus and Kawasaki disease are the most common disorders associated with a strawberry tongue. The “strawberry-like” surface characteristics are caused by prominent lingual papillae. A white strawberry tongue is caused by fibrinous exudate overlying the tongue. Red strawberry tongues lack the fibrinous exudate.
38. What should parents look for in a sunscreen label?
The FDA has mandated updated language regarding sunscreen labeling. Parents should look for a sunscreen that is described as broad spectrum, which indicates that it will be effective against both ultraviolet A (UVA) and ultraviolet B (UVB) rays. A product with a sun protection factor (SPF) of 30 to 50 is preferred; the maximum allowable labeling is SPF 50 because higher does not afford significantly greater protection. The words sunblock and waterproof are no longer allowed on the label. Parents should look for water-resistant sunscreen; however, 80 minutes is the maximum time that a sunscreen will remain on the skin during water-based activities or with sweating. Advice should also include physical protection including wearing sun-protective clothing, hats, and sunglasses. Of note, a vitamin D supplement should be considered when practicing cautious sun protection, especially if insufficient vitamin D is being derived from a fortified diet.
ECZEMATOUS DISORDERS
39. What is the difference between eczema and atopic dermatitis?
The term eczema derives from the Greek word ekzein, which means to erupt: ek (ex) (out) plus zein (to boil). To most physicians, eczema is synonymous with atopic dermatitis, a chronic skin disease manifested by intermittent skin eruptions. Eczema is primarily a morphologic term used to describe an erythematous, scaling, inflammatory eruption with itching, edema, papules, plaques, vesicles, and crusts. There are other “eczematous eruptions” (nummular eczema, allergic contact dermatitis), but “garden variety” eczema is certainly the most common.
Atopic dermatitis is a broader allergic tendency with multiple dermal manifestations that are mostly secondary to pruritus. Atopic dermatitis has been called an “itch that rashes, not a rash that itches.” Its manifestations are dry skin, chronic and recurrent dermatitis, low threshold to pruritus, hyperlinear palms, eyelid pleats (Dennie-Morgan folds), pityriasis alba, and keratosis pilaris, among others.
40. What is the usual distribution of rash in atopic dermatitis?
• Infantile: Cheeks; chin; trunk; and extensor surfaces of extremities, knees, and elbows
• Childhood (age 2 to puberty): Neck; feet; wrists; and periorbital, antecubital, and popliteal fossae
• Adult: Neck, hands, feet, and antecubital and popliteal fossae
KEY POINTS: MAIN FEATURES OF ATOPIC DERMATITIS
1. Extensor surface involvement in infancy
2. Flexural surface involvement in older children
3. Lichenification with chronic scratching
4. Dennie-Morgan folds under eyes
5. Part of atopic triad: atopic dermatitis, asthma, and allergic rhinitis
6. Decreased innate immunity in skin leads to increased susceptibility to bacterial and viral skin infections
41. Describe the five key battle plans to treat atopic dermatitis
1. Reduce pruritus. Topical corticosteroids and bland emollients help reduce pruritus. Oral antihistamines may also be used for their sedative effect at night and may reduce pruritus.
2. Hydrate the skin. Emollients (petrolatum and fragrance-free ointments and creams) prevent the evaporation of moisture via occlusion and are best applied immediately after bathing, when the skin is maximally hydrated, to “lock in” moisture. A “soak and smear” protocol is advisable in refractory cases.
3. Reduce inflammation. Topical steroidsa are invaluable as anti-inflammatory agents and can hasten the clearing of eruptions that are erythematous (inflamed). Medium-strength corticosteroids can be used on areas other than the face and occluded regions (diaper area); low-strength steroids (e.g., 1% hydrocortisone) may be used in these thin-skinned areas for limited periods of time. Topical immunomodulators, such as topical tacrolimusb and pimecrolimus, are approved for the intermittent treatment of moderate to severe atopic dermatitis in children 2 years old and older. However, their long-term side effects have not been fully evaluated.
4. Control infection. Superinfection with Staphylococcus aureus is extremely common. First- generation cephalosporins such as cephalexin are the usual antibiotics of choice for infected atopic dermatitis. Dilute bleach baths are sometimes recommended 2 to 3 times per week to reduce staphylococcal colonization.
5. Avoid irritants. Gentle fragrance-free soaps and shampoos should be used; wool and tight synthetic garments should be avoided; tight nonsynthetic garments may help minimize the “itchy” feeling; consider furniture, carpeting, pets, and dust mites as possible irritants and/or trigger factors.
Eichenfield LF, Tom WL, Chamlin SL, et al: Guidelines of care for the management of atopic dermatitis: section 1. Diagnosis and assessment of atopic dermatitis, J Am Acad Dermatol 70:338–351, 2014.
Eichenfield LF, Tom WL, Berger TG, et al: Guidelines of care for the management of atopic dermatitis: section 2. Management and treatment of atopic dermatitis with topical therapies, J Am Acad Dermatol 71:116–132, 2014. Sidbury R, Davis DM, Cohen DE, et al: Guidelines of care for the management of atopic dermatitis:
section 3. Management and treatment with phototherapy and systemic agents, J Am Acad Dermatol 71:327– 349, 2014.
42. Why is there a black box warning on topical calcineurin inhibitors?
The long-term side effects of chronic use are still under investigation. A black box warning is in place based on concerns regarding systemic absorption and the associated increased cancer risk in patient populations in which long-term systemic administration has occurred, such as organ transplant recipients. Patients should be instructed about the importance of sun protection while using topical immunosuppressive medications such as topical calcineurin inhibitors.
Thaçi D, Salgo R: Malignancy concerns of topical calcineurin inhibitors for atopic dermatitis: facts and controversies,
Clin Dermatol 28:52–56, 2010.
43. Why shouldn’t fluorinated (halogenated) and other potent topical steroids be used on the face?
• Facial skin is thinner and therefore percutaneous absorption is higher.
• Telangiectasias or spider veins can occur.
• Cutaneous atrophy can occur.
• Perioral dermatitis or poststeroid rosacea can occur with rebound symptoms that are worse than the original rash.
44. Is there a genetic basis for atopic dermatitis?
It is likely that both genetic and environmental factors play a role. Susceptibility to atopic dermatitis is found in patients with mutations in filaggrin, an epithelial protein that cross-links keratin and serves to waterproof the epidermis, or outer layer of skin. Many children with atopic dermatitis have a family history of atopy. If one parent has an atopic diathesis, 60% of offspring will be atopic; if two parents do, 80% of children are affected. Monozygotic twins are often concordant for atopic disease.
Palmer CN, Irvine AD, Terron-Kwiatkowski A, et al: Common loss-of-function variants of the epidermal barrier protein filaggrin are a major predisposing factor for atopic dermatitis, Nat Genet 38:441–446, 2006.
45. Are there consistent immunologic alterations in children with atopic dermatitis? Humoral changes include elevated immunoglobulin E levels and a higher-than-normal number of positive skin tests (type I cutaneous reactions) to common environmental allergens. Cell-mediated abnormalities have been found only during acute flares of the dermatitis; these include mild to moderate depression of cell-mediated immunity, a 30% to 50% decrease in lymphocyte-forming E-rosettes, decreased phagocytosis of yeast cells by neutrophils, and chemotactic defects of polymorphonuclear and mononuclear cells. Innate immunity of the skin is altered with lower levels of antimicrobial peptides and increased susceptibility to bacterial and viral cutaneous infections.
Eichenfield LF, Tom WL, Chamlin SL, et al: Guidelines of care for the management of atopic dermatitis: section 1. Diagnosis and assessment of atopic dermatitis, J Am Acad Dermatol 70:338–351, 2014.
46. What is the role of filaggrin mutations in the development of atopic dermatitis? The filaggrin gene encodes for an epidermal protein (filaggrin, short for filament-aggregating protein) that is abundantly expressed in the outer layer of the epidermis. Filaggrin is essential for epidermal homeostasis and maintenance of skin barrier functions, as well as retention of water. About 10% of individuals of European ancestry are at least heterozygous carriers for the gene, which results in a 50% decrease in the expressed filaggrin protein. This group is more likely to develop eczema of increased severity.
Irvine AD, McLean WHI, Leung DYM: Filaggrin mutations associated with skin and allergic diseases, N Engl J Med
365:1315–1327, 2011.
47. What other skin conditions mimic atopic dermatitis?
• Seborrheic dermatitis
• Scabies
• Psoriasis
• Tinea capitis
• Lichen simplex chronicus
• Acrodermatitis enteropathica
• Contact dermatitis
• Langerhans cell histiocytosis
• Xerotic eczema (dry skin)
• Immunodeficiency disorders (e.g., Wiskott-Aldrich syndrome, hyperimmunoglobulin E syndrome, severe combined immunodeficiency)
• Nummular eczema
• Metabolic disorders (e.g., phenylketonuria, essential fatty acid deficiency, biotinidase deficiency)
48. What is the “atopic march”?
Approximately half of infants with atopic dermatitis will develop asthma, and two thirds will develop allergic rhinitis. Thus, the one condition in infancy marches toward others. Currently under study are ways to interrupt this progression.
Spergel JM, Paller AS: Atopic dermatitis and the atopic march, J Allergy Clin Immunol 112: S118–S127, 2003.
49. What features help differentiate seborrheic from atopic dermatitis during infancy?
See Table 4-1.
Table 4-1. Seborrheic Dermatitis Versus Atopic Dermatitis
CHARACTERISTICS SEBORRHEIC DERMATITIS ATOPIC DERMATITIS
Color Salmon Pink or red (if inflamed)
Scale Yellowish, greasy White, not greasy
Age Infants <6 months or adolescents May begin at 2-12 months and continue through childhood
Itching Not present May be severe
Distribution Face, postauricular scalp, axillae, and groin Cheeks, trunk, and extensors of extremities
Associated features None Dennie-Morgan folds, allergic shiners, hyperlinear palms
Lichenification None May be prominent
Response to topical steroids Rapid Slower
50. How should parents cope with cradle cap?
Seborrheic dermatitis of the scalp, also known as “cradle cap,” occurs during infancy and presents as a yellow, greasy, scaling adherent rash on the scalp that may extend to the forehead, eyes, ears, eyebrows, nose, and the back of the head. It appears during the first few months of life and generally resolves in several weeks to a few months. Treatment includes the application of mineral oil followed by shampooing with a mild antidandruff shampoo containing selenium sulfide.a Parents should be cautioned to take extra care when washing the scalp because these shampoos may irritate the infant’s eyes. A mild-potency topical steroida such as hydrocortisone (1% to 2.5%) may be needed for persistent areas. Families should be advised not to scrub or pick off the scale because the underlying skin is often tender and inflamed.
51. What condition causes bumps on the cheeks, upper arms, and thighs?
Keratosis pilaris. Associated both with atopic dermatitis and ichthyosis vulgaris, this condition runs in families and is asymptomatic. It is characterized by spiny follicular papules, giving involved areas a “chicken skin” or “gooseflesh” feel. Usual treatment is with bland emollients or emollients that contain a mild peeling agent, such as lactic acid,a salicylic acid,a or an alpha hydroxy acid preparation.a
52. What are the causes of irritant contact diaper rash?
A variety of local factors are involved. Diapers contribute to the chafing of the skin and the prevention of moisture evaporation, thus increasing epidermal hydration and permeability to irritants and mechanical injury. Proteolytic enzymes in urine and stool and ammonia generated from urine irritate chafed skin. Seasoned pediatricians will advise that alcohol-based diaper wipes also feed the flames of diaper rash.
53. What features of diaper rash suggest more sinister diseases?
• Marked tenderness, rapid onset (staphylococcal scaled skin syndrome)
• Deep ulcerations, vesicles (herpes simplex)
• Beefy red, erosive, extensive lesions (particularly intertriginous) that are poorly responsive to topical steroids and antifungals (Langerhans cell histiocytosis, acrodermatitis enteropathica, immunodeficiency states)
• Extensive and severe lesions with pungent odor (abuse or neglect with infrequent changing)
Boiko S: Making rash decisions in the diaper area, Pediatr Ann 29:50–56, 2000.
54. Are topical steroid/antifungal preparations useful for treating children with diaper dermatitis?
Most diaper dermatitis is usually diagnosed as either irritant contact dermatitis or candidal dermatitis. Irritant diaper dermatitis responds well to very-low-potency topical corticosteroidsa (as a result of their anti-inflammatory properties) and a topical barrier such as zinc oxide ointment. Candidiasis of the diaper area responds well to topical antifungal preparations; rarely, an oral anticandidal medication is necessary.a In both types of diaper dermatitis, frequent diaper changes, exposure to air, and avoidance of excessive moisture are helpful. Combination preparations containing both antifungal and corticosteroid medications are not recommended to treat diaper dermatitis
because the strength of the steroid component in these products is usually too high for use in the diaper area.
Kazaks EL, Lane AT: Diaper dermatitis, Pediatr Clin North Am 47:909–920, 2000.
55. Which dietary deficiencies may be associated with an eczematous dermatitis? Deficiencies in zinc, biotin, essential fatty acids, and protein (kwashiorkor) may be associated with eczematous dermatitis.
56. What are the two main types of contact dermatitis?
Irritant and allergic. Irritant contact dermatitis arises when agents such as harsh soaps, bleaches or acids have direct toxic effects when they come into contact with the skin. Allergic contact dermatitis is a T-cell mediated inflammatory immune reaction that requires sensitization to a specific antigen.
57. What type of agents can cause allergic contact dermatitis in children? Allergic contact dermatitis can occur in all age groups, but it is often underrecognized in pediatric patients. Sensitizers include plant resins (poison ivy, sumac, or oak); nickel in jewelry, metal snaps, and belts; topical neomycin ointment; preservatives (formaldehyde releasers); fabric dyes; and materials used in shoes, including adhesives, rubber accelerators, and leather tanning agents. Of note, allergic contact dermatitis due to metals in mobile phones and other portable electronic devices, especially nickel and chromium, has been on the rise in recent years.
Jacob SE, Admani S: iPad-increasing nickel exposure in children, Pediatrics 134:e580–e582, 2014.
Richardson C, Hamann CR, Hamann D, et al: Mobile phone dermatitis in children and adults: a review of the literature,
Pediatr Allergy Immunol Pulmonol 27:60–69, 2014.
58. When does the rash in poison ivy appear relative to exposure?
Poison ivy, or rhus dermatitis, is a typical delayed hypersensitivity reaction. The time between exposure and cutaneous lesions is usually 2 to 4 days. However, the eruption may appear as late as a week or more after contact in individuals who have not been previously sensitized (this explains why lesions continue to erupt after the initial “outbreak” of rash).
59. Are the vesicles in poison ivy contagious?
No. The contents of blisters do not contain the allergen. Washing the skin removes all surface oleoresin and prevents further contamination.
60. What is the “id” reaction?
Your superego will be stroked if you identify the “id” reaction in a confusing dermatologic case. This reaction is the generalization of a local inflammatory dermatitis (e.g., contact dermatitis, tinea capitis following treatment) to sites that have not been directly involved with the offending agent. The exact mechanism remains unclear, but it may be immune-complex mediated.
61. How does the vehicle used in a dermatologic preparation affect therapy?
In general, acute lesions (moist, oozing) are best treated with aqueous, drying preparations. Chronic, dry lesions fare better when a lubricating, moisturizing vehicle is used. As a rule, any vehicle that enhances hydration of the skin enhances the percutaneous absorption of topical medications (most of which are water soluble). Thus, in preparations of equal concentration, the potency relationship is ointment> cream> gel> lotion. See Table 4-2.
Table 4-2. Vehicles Used in Dermatologic Preparations
Drying Vehicles
Lotion: A suspension of powder in water; therapeutic powder remains after aqueous phase evaporates; useful in hairy areas, particularly the scalp
Gel: Transparent emulsion that liquifies when applied to skin; most useful for acne preparations and tar preparations for psoriasis
Pastes: Combination of powder (usually cornstarch) and ointment; stiffer than ointment
Moisturizing Vehicles
Creams: Mixture of oil in a water emulsion; more useful than ointments when environmental humidity is high and in naturally occluded areas; less greasy than ointment
Ointments: Mixture of water in an oil emulsion; also has an inert petroleum base; longer lubricating effect than cream
FUNGAL INFECTIONS
62. What are useful methods for diagnosing tinea infections?
Although the microscopic examination of potassium hydroxide (KOH) preparations is employed in the search for hyphae, the use of dermatophyte test medium (DTM) is reliable, simple, inexpensive, and more definitive. Samples from hair, skin, or nails are obtained by scraping with a scalpel, cotton-tipped applicator, or toothbrush (the latter especially for tinea capitis), and these are inoculated directly onto the test medium. After approximately 1 to 2 weeks, a color change from yellow to red in the agar surrounding the dermatophyte colony indicates positivity. If the most definitive diagnosis is needed, culture on Sabouraud medium is the test of choice.
KEY POINTS: MAIN FEATURES OF TINEA CAPITIS
1. Scaly alopecia
2. Black-dot hairs often observed
3. Associated with posterior cervical adenopathy
4. Potassium hydroxide test often positive
5. Diagnosis confirmed by positive fungal culture
6. Most common cause: Trichophyton tonsurans
63. How does one differentiate between irritant diaper dermatitis and candidal diaper dermatitis?
Both types of dermatitis often present together. Classic irritant diaper dermatitis involves the skin, which contacts the diaper with sparing of the protected skin folds. Candidal dermatitis, on the other hand, favors skin folds such as the inguinal folds and intragluteal cleft. In clinical practice, candidal infection is a common infection that can be precipitated by the compromise of the cutaneous barrier seen in irritant dermatitis. With candida infection, one typically sees confluent, beefy red plaques involving the groin creases. Scale and satellite papules or pustules are commonly seen at the periphery of the plaque. The diagnosis can be made clinically and with a KOH preparation and yeast culture. Treatment comprises a topical anticandidal agent, such as clotrimazolea and use of a thick barrier cream, such as zinc oxide. A few days of a low potency corticosteroid ointment may reduce the erythema significantly. Remember to consider other causes such as seborrheic dermatitis, psoriasis, acrodermatitis enteropathica, Langerhans cell histiocytosis or immunodeficiency for chronic and refractory diaper dermatitis.
64. Does a scaly scalp and swollen glands qualify a patient to initiate griseofulvin? No. In one study, scalp scaling and cervical adenopathy were more often caused by seborrheic or atopic dermatitis than tinea capitis.
Williams JV, Eichenfield LF, Burke BL, et al: Prevalence of scalp scaling in prepubertal children, Pediatrics 115:e1–e6, 2005.
65. Why is it necessary to culture for tinea capitis?
Tinea capitis can be caused by a variety of dermatophyte fungal organisms, and over the last decade, resistance to commonly used treatments (griseofulvin) has been noted. Children with tinea capitis are requiring longer courses of treatment and higher doses of medication to eradicate the fungal infection. Moreover, other conditions (e.g., alopecia areata, psoriasis of the scalp) may be confused with tinea capitis. Therefore, just like for other pediatric infections, it is important to document the type of infection with a culture so that proper treatment may be administered.
66. How can a culture be obtained if fungal culture medium is not available in the office? The simplest method is to take a cotton swab culturette and moisten it with water. Then, take the swab and rub it over the affected areas and all four quadrants of the scalp. The cotton swab can be used to directly inoculate the fungal culture media if you have it in the office or transported back to the laboratory for inoculation.
Friedlander SF, Pickering B, Cunningham BB, et al: Use of the cotton swab method in diagnosing tinea capitis, Pediatrics
104:276–279, 1999.
67. What are the clinical presentations of tinea capitis? Tinea capitis occurs more commonly in preadolescent children, boys, and in African-American children in the United States. It can present with a variety of morphologic characteristics including scalp scaling, alopecia, erythema, papules, pustules, “black dot” tinea, or a kerion (a boggy, tender mass). The “black dot” presentation occurs when the infected hair shaft breaks at the surface of the scalp, leaving a bald patch with black dots (or lighter dots, depending on hair color) (Fig. 4-6). Regional adenopathy is very common with inflammatory tinea.
Hubbard TW: Predictive value of symptoms in diagnosing childhood tinea capitis, Arch Pediatr Adolesc Med
153:1150–1153, 1999.
Figure 4-6. Black dot tinea. (From Schachner LA, Hansen RC, editors: Pediatric Dermatology, ed 3. Edinburgh, 2003, Mosby, p 1096.)
68. How should children with tinea capitis be treated? The dermatophytes (i.e., fungi) that cause tinea thrive deep in the hair shaft, beyond the reach of topical therapy alone. Recommended therapy includes systemic courses of griseofulvin or terbinafine. The choice of medication should be individualized based on culture results (griseofulvin remains treatment of choice for Microsporum species), cost, duration of therapy, and consideration of compliance. Oral griseofulvin (microsize or ultramicrosize preparation) has a long-term safety profile in children. It is given with fatty foods such as milk or ice cream to facilitate absorption. Terbinafine granules, which may be sprinkled onto nonacidic food, are FDA approved for the treatment of tinea capitis in children 4 years of age and older.
Moriarty B, Hay R, Morris-Jones R: The diagnosis and management of tinea, BMJ 345:e4380, 2012.
69. How should children who are receiving systemic medication for tinea capitis be monitored?
The incidence of hepatitis or bone marrow suppression from GRISEOFULVIn in children is rare. Children who are undergoing an acute course of treatment (6 to 8 weeks) do not need obligatory blood counts or liver function tests. However, a history of hepatitis or associated symptoms would warrant a pretreatment evaluation of liver function and intermittent monitoring. Resistance by tinea to griseofulvin is increasing, and higher, longer dosing may be needed to achieve clinical cure. For those rare cases in which
griseofulvin is going to be used for >2 months, one should consider obtaining complete blood counts and liver function tests on an every-other-month basis. When using terbinafine, a complete blood count
and hepatic function panel are recommended to be performed at baseline and patients should be warned/monitored for evidence of side effects such as hepatotoxicity. Rare cases of hepatic failure and bone marrow suppression have been reported.
70. What is a kerion?
A kerion is a fluctuant and tender mass that occurs in some cases of tinea capitis. It can be associated with alopecia, pustules, and purulent drainage. It is believed to be primarily an excessive inflammatory response to tinea, and thus, initial treatment consists of systemic antifungal agents, principally griseofulvin (>2 years) or terbinafine (> age 4 years), and selenium sulfide shampoo.a Prompt diagnosis and management is important to minimize the potential for permanent scarring. Short courses of oral
steroids can be considered in those lesions that are exquisitely painful.a
Honig PJ, Caputo GL, Leyden JJ, et al: Microbiology of kerions, J Pediatr 123:422–424, 1993.
71. What puts the “versicolor” in tinea versicolor?
A very common superficial disorder of the skin, tinea versicolor (also known as pityriasis versicolor) is caused by the yeast form of Malassezia furfur, known as Pityrosporum orbiculare and Pityrosporum OVALE. It appears as multiple macules and patches with fine scales over the upper trunk; arms; and occasionally, the face and other areas (Fig. 4-7). Lesions are “versatile” in color (i.e., light tan, reddish, or white) and may be “versatile” by season (i.e., lighter in summer and darker in winter as compared with surrounding skin). The yeast interferes with melanin production, possibly by the disruption of tyrosinase activity, at the involved sites. Diagnosis can be confirmed with a KOH preparation of a scraping from the involved skin, which has characteristic fungal hyphae and a grapelike spore pattern referred to as a “spaghetti and meatball” appearance. Dyspigmentation may persist for months after treatment.
72. How is tinea versicolor treated?
Figure 4-7. Tinea versicolor on the chest. (From Gawkrodger DJ: Dermatology: An Illustrated Colour Text, ed 3. London, 2002, Churchill LIVINGSTONE, p 38.)
• Selenium sulfide 2.5% lotion:a The shampoo or lotion is applied over the affected area overnight nightly during the first week, with decreasing frequency over the ensuing weeks. Monthly application may decrease recurrences.
• Ketoconazole 2% shampoo:a The shampoo is applied to wet skin and lathered. Patients are instructed to let the shampoo remain in place without washing for 3 to 5 minutes. Treatment is repeated for 1 to 3 days in a row. Monthly prophylactic treatments are suggested to prevent recurrence.
• Topical antifungal creams:a May be applied once to twice daily to limited areas. This is generally less favored in diffuse disease, which is commonly seen.
• Oral antifungal agents:a These treatments, which are sometimes effective after a single one-time dose, may be considered off label in severe diffuse or recalcitrant tinea versicolor in older children and adolescents. However, side effects, including liver toxicity, should be considered.
73. Which rashes resemble tinea pedis (athlete’s foot) in children?
• Dyshidrotic eczema: Erythema with microvesicles of interdigital spaces and lateral feet
• Contact dermatitis: Typically involves dorsum of feet and spares interdigital spaces
• JUVENILE plantar dermatosis: Glazed erythema and fissuring of toes and distal soles; often pruritic
• Pitted keratolysis: Small pits that may converge to superficial erosions on sole of foot; hyperhidrosis and malodor is common; associated with Corynebacterium species or Micrococcus sedentarius infections
HAIR AND NAIL ABNORMALITIES
74. How fast does hair grow?
Hair grows about 1 cm per month.
75. On what parts of the skin is hair not normally found?
Hair is not normally found on the palms, soles, genitalia, and medial/lateral aspects of toes and fingers.
76. What causes sparse or absent hair in children?
• Congenital localized: Nevus sebaceous (or sebaceous), aplasia cutis, incontinentia pigmenti, focal dermal hypoplasia, intrauterine trauma, infection (e.g., herpes)
• Congenital diffuse: Loose anagen syndrome, Menkes syndrome, trichoschisis, genetic syndromes (e.g., ectodermal dysplasia, lamellar ichthyosis, Netherton syndrome)
• Acquired localized: Tinea capitis, alopecia areata, traction alopecia, traumatic scarring
(e.g., trichotillomania), androgenic alopecia, Langerhans cell histiocytosis, lupus erythematosus
• Acquired diffuse: Telogen effluvium, anagen effluvium, acrodermatitis enteropathica, endocrinopathies (e.g., hypothyroidism)
Datloff J, Esterly NB: A system for sorting out pediatric alopecia, Contemp Pediatr 3:53–56, 1986.
77. Which kind of alopecia simply requires a change in hairstyle as the treatment? Traction alopecia. This condition is due to styling with tight braids or ponytails that create tension on the hair shaft. This process damages the area, which serves as the source of new cells for the hair follicle. An erythematous papular or papulopustular reaction may be seen. Treatment involves loose hair styles, avoiding chemical processing or heat treatments. With appropriate changes in hairstyling early on, the prognosis is excellent. However, if the process continues, it may result in scarring alopecia.
Castrelo-Soccio, L: Diagnosis and management of alopecia in children, Pediatr Clin North Am 61:427–442, 2014.
78. How can alopecia areata be differentiated clinically from tinea capitis?
In tinea capitis, the fungal organism invades the hair shaft but is also present in the epidermis (the top layer of the skin). There are usually changes of scaling and inflammatory lesions that are intermingled with black dots representing broken hairs. In alopecia areata, the scalp is smooth, although patches may be pink to peach colored (Fig. 4-8). Some hairs within the patch may have a tapered appearance, with
Figure 4-8. Well-demarcated, hairless patches of alopecia areata.
the wider end distally and a thinner end at the base of the scalp (i.e., the “exclamation point hair”). There is no lymphadenopathy in patients with alopecia areata, but this is not uncommon in patients with tinea capitis. The gold standard for diagnosis of tinea is a positive fungal culture.
79. What are reported as poor prognostic indicators for recovery of hair in patients with alopecia areata?
• Atopy
• Presence of other immune-mediated disease (e.g., thyroid disease, vitiligo)
• Family history of alopecia areata (about 25% of patients)
• Young age at onset
• Longer duration of active disease
Gilhar A, Etzioni A, Paus R: Alopecia areata, N Engl J Med 366:1515–1525, 2012. Uchiyama M, Egusa C, Hobo A, et al: Multivariate analysis of prognostic factors in patients with rapidly progressive alopecia areata, J Am Acad Dermatol 67:1163–1173, 2012.
80. What are treatments for alopecia areata?
Treatment is based on the extent of disease: patchy, totalis (loss of all scalp hair), or universalis
(loss of all body hair). Although the cause is unknown, alopecia areata is generally considered to be a T-cell-mediated autoimmune disorder. Therefore, treatments are directed at suppressing the immune response around the hair follicle. Topical or intralesional corticosteroids are the mainstays of therapy.a Systemic corticosteroids are rarely used chronically because of side effects.a Anthralin, as an irritant, or other topical sensitizers are designed to cause a mild dermatitis and theoretically alter local immunity to promote hair regrowth.a Other systemic immunosuppressive treatments have been tried in adult patients with alopecia areata; however, because of the potential for side effects, they are not typically employed in children. The option of not treating must be reviewed with the family. Support groups should be offered, and many patients have had improved self-esteem after being fitted with a hair prosthesis.
Castrelo-Soccio, L: Diagnosis and management of alopecia in children, Pediatr Clin North Am 61:427–442, 2014. Children’s Alopecia Project: www.childrensalopeciaproject.org. Accessed Nov. 17, 2014.
National Alopecia Areata Foundation: www.naaf.org. Accessed Nov. 17, 2014.
81. Are most hairs growing or resting? Most infants and children have about 90% of scalp hair in the growing (anagen) state and about 10% in the resting (telogen) state. On average, a single scalp hair will grow for about 3 years, rest for 3 months, and then, upon falling out, be replaced by a new growing hair.
82. You are evaluating a healthy 4-year-old with fine, sparse hair who has never had a haircut. What condition do you suspect?
Loose anagen syndrome is typically seen in 2- to 5-year-old blond girls, but may also present in children with darker hair. The hair is of variable lengths and is easily pulled from the scalp. A microscopic examination of a few pulled hairs reveals predominance of anagen hair bulbs with ruffled cuticles. There are no associated nail, skin, or teeth findings in loose anagen syndrome. There is no treatment, but gentle hair styling should be encouraged. Fortunately, the condition tends to improve over time.
Dhurat RP, Deshpande DJ: Loose anagen hair syndrome, Int J Trichology 2:96–100, 2010.
83. What is the likely diagnosis in a child who develops diffuse hair loss 3 months after major surgery? Telogen effluvium. This is the most common cause of acquired diffuse hair loss in children. In a healthy individual, most hairs are present in a growing (anagen) phase. After a physical or emotional stress such as a significant fever, illness, pregnancy, birth, surgery, or large weight loss, a large number of scalp hairs can convert to the resting (telogen) phase. About 2 to 5 months after the stressful event, the hair begins to shed, at times coming out in large clumps. The condition is temporary and usually does not produce a loss of more than 50% of the hair. When the hair roots are examined, there is a characteristic lighter-colored root bulb, which characterizes a telogen hair. The hair loss can continue for 6 to 8 weeks, at which time new, short, regrowing hairs should be visible. The differential diagnosis for telogen effluvium includes nutritional deficiencies and abnormal thyroid function.
Anagen effluvium, the loss of growing hairs, is most commonly seen during radiation and chemotherapy treatments for cancer.
84. What puzzling cause of asymmetric hair loss in a child will sometimes cause an intern to pull his or her hair out?
Trichotillomania is hair loss as a result of self-manipulation, such as rubbing, twirling, or pulling. Hair loss is asymmetric. The most common physical finding is unequal hair lengths in the same region without evidence of epidermal changes of the scalp. Parents often do not observe the causative behavior, and convincing them of the likely diagnosis may take some effort. Behavior modification is often the first line of treatment. In younger children, the hair pulling behavior usually resolves; in older children it may persist and require psychiatric referral for additional management. Rarely, a child will swallow the hair and develop vomiting because of the formation of a gastric trichobezoar (hairball).
85. What causes green hair?
Children with blond or light-colored hair can develop green hair after long-term exposure to chlorinated swimming pools. It is the result of the incorporation of copper ions into the hair matrix. Over-the-counter chelating shampoos are available for prevention and treatment.
86. How should ingrown toenails be managed?
Soaks, open-toed sandals, properly fitting shoes, topical or systemic antibiotics, incision and drainage, or surgical removal of the lateral portion of the nail may all be used. Control is best obtained by letting the nail grow beyond the free end of the toe. Proper instruction on nail care, including straight rather than arc trimming, is mandatory.
87. Which pathogens are responsible for paronychia?
Acute paronychia (inflammation of the nail fold, usually with abscess formation) is most commonly caused by Staphylococcus aureus. The proximal or lateral nail fold becomes intensely erythematous and tender. If a collection of pus develops at this site, it should be incised and drained. The treatment of acute paronychia includes the oral administration of antistaphylococcal antibiotics.
Chronic paronychia is most often caused by Candida albicans and often involves a history of chronic water exposure (e.g., dishwashing, thumb sucking). Although rarely inflamed, there is edema of the nail folds and separation of the folds from the nail plate. The nails may become ridged and develop a yellow-green discoloration. A bacterial culture may reveal a variety of gram-positive and gram-negative organisms. Therapy includes topical antifungal agents and avoidance of water. There is no place for griseofulvin in the treatment of chronic paronychia.
88. A healthy 7-year-old child who develops progressive yellowing and increasing friability of all nails over a period of 12 months likely has what condition?
Twenty-nail dystrophy (trachyonychia). The progressive development of rough nails with longitudinal grooves, pitting, chipping, ridges, and discoloration occurring in isolation in school-aged children has been given this name, although not all nails need be involved. The etiology remains unclear, and a majority of cases resolve spontaneously without scarring. The nail changes, however, may be associated with other conditions, such as alopecia areata, lichen planus, psoriasis, and atopic dermatitis.
89. What nail change may follow hand, foot, and mouth disease (HFMD) several weeks after the other hand and foot changes have resolved? Onychomadesis is separation of the proximal nail plate from the nail bed, which is believed to be caused by arrest of nail growth. It is most commonly associated with illness and appears several weeks afterwards. There are now numerous reports of onychomadesis following HFMD caused by Coxsackie virus.
Chu DH, Rubin AI: Diagnosis and management of nail disorders in children, Pediatr Clin North Am 61:293–308, 2014.
INFESTATIONS
90. How do lice differ?
• Pediculosis capitis (head lice): Pediculus capitis, the smallest and most common of the three human lice, is an obligate human parasite. Spread occurs directly by contact with an infected individual or indirectly through the use of shared combs, brushes, or hats. Infestation is more common in fine versus coarse hair types.
• Pediculosis corporis (body lice): Pediculus humanus, the largest (2 to 4 mm) of the three types, is usually associated with poor hygiene. It does not live on the body but instead in the seams of clothing. It can be a vector for other diseases, such as epidemic typhus, trench fever, and relapsing fever.
• Pediculosis pubis (pubic lice): Phthirus pubis is also known as the crab louse because it is a broad insect with legs that look like claws. It is sometimes mistaken for a brown freckle. Acquisition is primarily through sexual contact.
91. What are the clinical findings of head lice infestation? Scalp pruritus is most common, but many children are asymptomatic. A search for lice should be made in any school-aged child presenting with scalp itching. Nits (lice eggs) are found in greatest density on the parietal and occipital areas.
92. How is the diagnosis of head lice made?
On physical examination, an actual louse (wingless, grayish insect about 3 to 4 mm) may be difficult to find, although one should easily be able to find nits. The nits are first attached to the hair close to
the surface of the scalp and are oval and flesh-colored (Fig. 4-9). They are not easily removed from the hair shaft (as compared with hair casts, dandruff, and external debris). Over-diagnosis of head lice is common. Microscopic evaluation of the suspected nit can confirm the diagnosis. When the louse emerges, the empty egg case, or nit, appears white in color and remains firmly attached to the hair shaft as the hair grows out (see Fig. 4-9).
Pollack RJ, Kiszewski AE, Spielman A: Overdiagnosis and consequent mismanagement of head louse infestations in North America, Pediatr Infect Dis J 19:689–693, 2000.
Figure 4-9. Viable head louse egg (right) and hatched empty nit (left) attached to a child’s hair. (From Schachner LA, Hansen RC, editors: Pediatric Dermatology, ed 3. Edinburgh, 2003,
Mosby, p 1143.)
93. What types of treatment are available for head lice?
• Permethrin: 1% and 5% (Nix, Elimite)
• Pyrethrins: (RID, A-200, R&C) (Resistance is increasing in these over-the-counter products.)
• Malathion: 0.5% (Ovide is FDA-approved for the treatment of head lice in children 6 years and older; it is contraindicated in children under 2 years of age.)
• 5% Benzyl Alcohol Lotion: (Ulesfia is FDA-approved for treatment of head lice in children 6 months of age and older.)
• Asphyxiants: Examples: petroleum jelly (Vaseline), mayonnaise, olive oil (These have questionable efficacy and are messy!)
• Ivermectin: 0.5% lotion (Sklice is FDA-approved for treatment of head lice in children 6 months of age and older.)
• Lindane: 1% (Kwell is not recommended given “black box warning” regarding serious neurotoxicity.)
• Nit picking: (See question 95.)
Pariser DM, Meinking TL, Bell M, et al: Topical 0.5% ivermectin lotion for treatment of head lice, N Engl J Med
367:1687–1693, 2012.
Frankowski BL, Bocchini JA Jr, Council on School Health and Committee on Infectious Diseases: Head lice, Pediatrics
126:392–403, 2010.
94. What precautions should be taken before prescribing malathion 0.5% lotion (Ovide) for head lice?
Malathion, a weak organophosphate cholinesterase inhibitor, is an approved prescription topical treatment for resistant head lice and their eggs. It is approved for use in children 6 years of age. It is contraindicated for neonates and infants. Because it is flammable, malathion should never be used near an open flame or heat source. It should be used in a well-ventilated area given its odor, and precautions also include increased absorption through open sores.
95. Should parents nit pick?
Once an infestation of lice has been properly treated, the nits are not viable or contagious. Despite this, many schools will not allow children with nits to attend even though this nit-free policy has not been shown to be of benefit for controlling outbreaks. Increasing resistance to therapy may make removal more important to avoid diagnostic confusion. Manual removal (nit picking) is the most effective method; however, it is time consuming and tedious. Fine-toothed combs, such as the LiceMeister comb (available through the National Pediculosis Association [www.headlice.org]) or other fine-toothed veterinary combs, aid in the removal.
96. How is a skin scraping for scabies or “scabies prep” done?
Because the highest percentage of mites are usually concentrated on the hands and feet, the web spaces between digits are the best places to look for the characteristic linear burrows. Moisten the skin with alcohol or mineral oil, scrape across the area of the burrow with a small, rounded scalpel blade (e.g., No. 15 or blunt-edged Fomon blade), and place the scrapings on a glass slide with a drop of KOH (or additional mineral oil, if used) and a cover slip. Burrows, if unseen, can be more precisely localized by rubbing a washable felt-tip marker across the web space and removing the ink with alcohol (called the burrow ink test). If burrows are present, ink will penetrate through the stratum corneum and outline the site. Under the microscope, mites, eggs, and/or scybala (mite feces) may be seen (Fig. 4-10).
Figure 4-10. Scabies mite and eggs. (From Gates RH, editor: Infectious Disease Secrets, ed
2. Philadelphia, 2003, Hanley & Belfus, p 356.)
97. What treatment eliminates the scabies’ babies? The treatment of choice for treating scabies is permethrin 5% cream (Elimite, Acticin). It may be used in children as young as 2 months old with a low risk of neurotoxicity.a It is more effective and preferred over Lindane, which has a much higher potential risk of neurotoxicity.
Permethrin cream is applied from the neck to the toes at night with removal after 8 to
14 hours by bathing or showering. The scalp is also treated in infants and younger children without a full head of hair. Retreatment in 1 week is recommended. Physicians must make patients aware of the fact that lesions and pruritus may linger for at least 2 weeks after effective therapy.
Antihistamines and low-potency topical steroids may help control symptoms.a It must be stressed that all family members and close contacts should be treated simultaneously.
Gunning K, Pippitt K, Kiraly B, et al: Pediculosis and scabies: treatment update, Am Fam Physician
86:535–541, 2012.
98. Which areas of the body are more commonly involved in scabies in younger children compared with adults?
Infants and children: axillae with nodules and scalp, face, soles, and dorsal foot
Adults: interdigital
Boralevi F, Diallo A, Miquel J, et al: Clinical phenotype of scabies by age, Pediatrics 133:e910–e916, 2014.
99. In what conditions is the “breakfast, lunch, and dinner” sign noted?
This is a tongue-in-cheek reference to the tendency of an insect to move from site to site for a meal. A typical appearance is a series of linear pink or urticarial papules each with a central pinpoint punctum that occur in response to the bite of a bed bug (Cimex lectularius) or other crawling insect (Fig. 4-11).
Figure 4-11. Bed bug bites with erythematous wheals or papules that may itch. They frequently attack exposed areas, especially at night. (Callen JP, Greer KE, Paller AS, et al: Color Atlas of Dermatology, ed 2. Philadelphia, 2000, WB Saunders, p 67.)
100. Will getting a new mattress ensure that the bed bugs won’t bite?
No, infestations are not limited to the mattress. Other furniture, clutter in the room, and cracks and crevices in the wall or floor may also harbor bedbugs. They are then attracted to the warm moist carbon dioxide around the sleeping child in the dark of night.
NEONATAL CONDITIONS
101. What are the most common birthmarks?
• Salmon patches (nevus simplex, vascular stains) are faint, pink-red, macular patches composed of distended dermal capillaries that are found on the glabella, eyelids, and the nape of the neck. They are seen in 70% of white infants and 60% of black infants. Although they usually fade, they may persist indefinitely, becoming more prominent during crying.
• Mongolian spots (dermal melanosis) are blue-black macules that are found on the lumbosacral area and occasionally on shoulders and backs. They are seen in 80% to 90% of babies with darker skin types but 10% of white fair-skinned infants. Most of these spots fade by 2 years of age and disappear by age 10.
102. How should pustular lesions be evaluated in the newborn period? It is very important to rule out infectious etiologies, because some may be life-threatening. The purulent material should be evaluated with a Gram stain, potassium hydroxide (KOH), Tzanck preparation, and bacterial and viral cultures. A Wright stain will reveal the presence of neutrophils or eosinophils.
See Table 4-3.
Table 4-3. Common Neonatal Papular Lesions
CHARACTERISTICS NEONATAL CEPHALIC PUSTULOSIS
MILIA ERYTHEMA TOXICUM
Distribution Face Face and other areas Face, trunk, and extremities
Appearance Papule or pustule Yellow or white papule Yellow or white papule
Erythematous Yes No Yes
Contents on smear PMNs Keratin+ sebaceous material Eosinophils
Incidence Occasional 40-50% of term infants 30-50% of term infants
Course Last several months Disappear in 3-4 weeks Disappear in 2 weeks
PMNs ¼ Polymorphonuclear cells.
103. What is the differential diagnosis of vesicles or pustules in the newborn?
See Table 4-4.
Table 4-4. Differential Diagnosis of Vesicles in the Newborn
NONINFECTIOUS INFECTIOUS
Miliaria Candidiasis
Erythema toxicum Staphylococcal folliculitis/impetigo
Transient neonatal pustular melanosis Herpes simplex
Benign cephalic pustulosis (neonatal acne) Congenital syphilis
Infantile acropustulosis Varicella
Incontinentia pigmenti Bacterial sepsis
Langerhans cell histiocytosis Scabies
Adapted from: Frieden IJ, Howard R: Vesicles, pustules, bullae, erosions and ulcerations. In Eichenfield LF, Frieden IJ, Esterly NB, editors: Textbook of Neonatal Dermatology, ed 2. Philadelphia, 2008, WB Saunders, pp 131–158.
104. What is the medical significance of cutis marmorata?
Cutis marmorata is the bluish mottling of the skin often seen in infants and young children who have been exposed to low temperatures or chilling. The reticulated marbling effect is the result of dilated capillaries and venules causing darkened areas on the skin, which disappears with warming. Cutis marmorata is of no medical significance and no treatment is indicated. However, persistent cutis marmorata is associated with trisomy 21, trisomy 18, and Cornelia de Lange syndromes. There is also a congenital vascular anomaly called cutis marmorata telangiectatic
congenita (CMTC) that has persistent purple reticulate mottling of the skin. In addition capillary malformations (port wine stains) may have a reticulated appearance and be mistaken for
cutis marmorata.
105. A healthy infant with scattered reddish nodules on the back skin most likely has what condition?
Subcutaneous fat necrosis consists of sharply circumscribed, indurated nodular lesions usually seen in healthy, term newborns and infants during the first few days to weeks of life. The stony hard areas of panniculitis are reddish to violaceous in color and are most often found on the cheeks, back, buttocks, arms, and thighs. Most lesions are self-limiting and require no therapy. However, occasionally they may extensively calcify and spontaneously drain with subsequent scarring. Remember that significant hypercalcemia may be present in a small number of patients. Therefore, a serum calcium level should be ordered whenever the disorder is suspected. It should be rechecked periodically until the condition resolves and for several months thereafter.
106. What should the family of a newborn with a yellow, hairless patch with a cobblestone texture be advised to do?
The lesion is likely a nevus sebaceous. This hamartomatous neoplasm usually presents as a yellow-pink hairless plaque on the scalp or face at the time of birth (Fig. 4-12) and is composed primarily of malformed sebaceous glands. Under the influence of androgens at puberty, the glands may hypertrophy and lead to the development of other neoplasms, most often
benign adnexal tumors. The exact risk of basal cell carcinoma development is controversial but generally low. Some experts advise excision during the preteen, prepubertal years. Careful
monitoring of the lesion for new growths or nonhealing ulcerations at all ages is advised, especially during adolescence.
Figure 4-12. Orange-yellow nevus sebaceous of the scalp. (From Kleigman RM, Stanton BF, Schor NF, et al: Nelson Textbook of Pediatrics, ed 19, Philadelphia, 2011, ELSEVIER Saunders, p 2235.)
107. What syndromes are associated with aplasia cutis congenita?
Aplasia cutis congenita (congenital absence of the skin) presents on the scalp as solitary or multiple well-demarcated ulcerations or atrophic scars. Of variable depth, the lesions may be limited to epidermis and upper dermis or occasionally extend into the skull and dura (Fig. 4-13). When a “hair collar” is present, an underlying connection with the CNS must be considered. Although most children with this lesion are otherwise normal without associated anomalies, other associations include epidermolysis bullosa, placental infarcts, teratogens, sebaceous nevi, and limb anomalies. Aplasia cutis is a feature of trisomy 13, 4p-, oculocerebrocutaneous syndrome and Adams-Oliver syndrome.
Figure 4-13. Healed aplasia cutis congenita with “hair collar sign.” (From Zitelli BJ, DAVIS HW: Atlas of Pediatric Physical Diagnosis, ed 5, Philadelphia, 2011, Mosby ELSEVIER, p 342.)
108. Describe the appearance and distribution of transient neonatal pustular melanosis.
Consisting of small vesicopustular lesions 2 to 4 mm in size, transient pustular melanosis
occurs in almost 5% of black and <1% of white newborns. It may be present at birth or appear shortly after birth. The lesions most often cluster on the neck, chin, palms, and soles, although they may occur on the face and trunk. The pustules rupture easily and progress to brown,
pigmented macules with a fine collarette of scale (Fig. 4-14). Microscopic examination of the
Figure 4-14. Pustular melanosis. (From Clark DA: Atlas of Neonatology. Philadelphia, 2000, WB Saunders, p 261.)
contents of the pustules reveals neutrophils with no organisms. There are no associated systemic manifestations, and the eruption is self-limited, although the hyperpigmentation may last for months.
109. Is erythema toxicum neonatorum really toxic?
Not in the least. Erythema toxicum is a common eruption composed of erythematous macules, papules, and pustules that occur in newborns, usually during the first few days of life. The lesions may start as irregular, blotchy, red macules, varying in size from millimeters to several centimeters.
They often develop into 1- to 3-mm, yellow-white papules and pustules on an erythematous base, giving a “flea-bitten” appearance. They occur all over the body except on the palms and soles, which are spared because the lesions occur in pilosebaceous follicles, which are absent on the palmar and plantar surfaces. The rash is less common in premature infants, with incidence proportional to gestational age and peaking at 41 to 42 weeks. Although it may be seen at birth, it is most common during the first 3 to 4 days of life and is occasionally noted as late as 10 days of life. Erythema toxicum usually lasts 5 to 7 days and heals without pigmentation. Other than the rash, the newborn appears healthy.
110. For academic (and billing) purposes, is it possible to be more scientific about the diagnosis of “prickly heat”?
The scientific name for this condition is miliaria rubra. It is due to sweat retention, and its clinical morphology is determined by the level at which sweat is trapped. Sweat trapped at a superficial level produces clear vesicles without surrounding erythema (sudamina or crystallina). Miliaria rubra (prickly heat, erythematous papules, vesicles, papulovesicles) is produced by sweat trapped at a deeper level. Pustular lesions (miliaria pustulosa) and even abscesses (miliaria profunda) are produced with sweat retention at the deepest of levels (infants rarely develop these types). With the advent of air conditioning, miliaria rarely occurs in newborn nurseries.
PAPULOSQUAMOUS DISORDERS
111. What diseases are associated with the Koebner reaction?
Koebnerization is a response to local injury whereby skin lesions are found at the sites of trauma (e.g., linear lesions at the sites of scratching). This is seen in patients with psoriasis (Fig. 4-15), as well as those with other conditions including lichen planus and flat warts.
Figure 4-15. Koebner phenomenon in psoriasis with linear plaques at site of excoriations. (From Cohen BA: Pediatric Dermatology, ed 2. St. Louis, 1999, Mosby, p 63.)
112. What is the typical pattern of lesions in childhood psoriasis?
Psoriasis presents as well-circumscribed, erythematous plaques with overlying white scale in children and adults. These occur on the scalp, elbows, knees (Fig. 4-16), sacrum, and genitalia. Psoriasis may also present with guttate (droplike) lesions over the trunk and extremities. These children may have group A beta hemolytic streptococcus infection as an underlying
precipitating factor.
Figure 4-16. Plaques of psoriasis on the knees. (From Gawkrodger DJ: Dermatology: An Illustrated Colour Text, ed 3. London, 2002, Churchill LIVINGSTONE, p 27.)
113. What percentage of children with psoriasis have nail involvement?
Nail changes, most commonly pitting, may be the only manifestation of psoriasis (Fig. 4-17). The reported incidence of nail pitting in children with psoriasis is as high as 40%. In a recent study, boys were found to have nail involvement more often than girls. Other nail changes include onycholysis (separation of the nail plate from nail bed at the distal margin) and thickening of the nail plate, often with white-yellow discoloration.
Mercy K, Kwasny M, Cordoro KM, et al: Clinical manifestations of pediatric psoriasis: results of a multicenter study in the United States, Pediatr Dermatol 30:424–428, 2013.
Figure 4-17. Nail pitting associated with psoriasis. (From Goldbloom RB: Pediatric Clinical Skills, ed 4. Philadelphia, 2011, ELSEVIER Saunders, p 228.)
114. A skin scale that easily bleeds on removal is characteristic of what condition?
The appearance of punctate bleeding points after removal of a scale is the Auspitz sign. It is seen primarily in psoriasis and is related to the rupture of capillaries high in the papillary dermis, near the surface of the skin.
115. How is the increased prevalence of childhood obesity associated with psoriasis?
Overweight and obesity are associated with higher likelihood of childhood psoriasis. In addition, adolescent patients with psoriasis have higher blood lipids. This highlights the importance of screening children with psoriasis for cardiovascular disease risk factors.
Koebnick C, Black MH, Smith N, et al: The association of psoriasis and elevated blood lipids in overweight and obese children, J Pediatr 159:577–583, 2011.
116. What are treatment modalities for psoriasis?
Various therapies have been used to treat psoriasis. The choice of treatment will depend on the extent of involvement, previous treatments, and the age of the patient. Topical treatments include topical corticosteroids, calcipotriene (a vitamin D analog), retinoids, and tar.a Other treatment modalities include UVB (typically narrow band UVB) phototherapy and, rarely, systemic retinoids and methotrexate.a Biologic agents such as etanercept have been used to treat widespread moderate-to-severe psoriasis in pediatric patients.a
Mercy K, Kwasny M, Cordoro KM, et al: Clinical manifestations of pediatric psoriasis: results of a multicenter study in the United States, Pediatr Dermatol 30:424–428, 2013.
Shah KN: Diagnosis and treatment of pediatric psoriasis, Am J Clin Dermatol 14:195–213, 2013.
117. What are the eight Ps of lichen planus?
• Papules: Usually 2 to 6 mm in diameter; often seen in a linear pattern as a result of the Koebner reaction
• Plaques: Commonly generated from a confluence of papules with exaggerated surface markings of the overlying skin (Wickham striae)
• Planar: Individual lesions, usually flat-topped
• Purple: Distinctly violaceous
• Pruritus: Often intensely itchy
• Polygonal: Borders of papules are often angulated
• Penis: Common site of involvement in children
• Persistent: Chronic, with remissions and exacerbations for up to 18 months
118. How is pityriasis rosea distinguished from secondary syphilis?
The distinction is often made with difficulty because both are primarily papulosquamous rashes. Pityriasis rosea classically consists of oval lesions that organize in parallel fashion on the trunk (the “Christmas tree” distribution) and are preceded in 40% to 80% of cases by a large annular
erythematous lesion (herald patch). Secondary syphilis lesions occur 3 to 6 weeks after the chancre, and compared with pityriasis rosea, they may have more involvement of the palms, soles, and mucous membranes and accompanying lymphadenopathy. However, because atypical presentations are common, testing for syphilis should be performed in any sexually active individual who is diagnosed with pityriasis rosea.
119. What is the treatment for pityriasis rosea?
Pityriasis rosea is a self-limited condition that usually resolves in 6 to 12 weeks. Therefore, treatment is often not required, unless there is significant pruritus or cosmetic disfigurement. A wide range of treatments are reported. Topical corticosteroidsa may help reduce pruritus, but they do not alter the course of the disease. UVB phototherapy results in clinical improvement in some individuals, and there are few reports to support the use of oral erythromycina to shorten the course. However, overall evidence supporting the use of these therapies is lacking.
Drago F, Broccolo F, Rebora A: Pityriasis rosea: an update with a critical appraisal of its possible herpesviral etiology,
J Am Acad Dermatol 61:303–318, 2009.
120. What is the likely diagnosis for a 5-year-old who presents with a linear array of recently acquired pink to hypopigmented papules on the arm?
Although the differential diagnosis may be broad, a common cause of this type of eruption is lichen striatus. This eruption is generally asymptomatic, does not require treatment, and lasts from weeks to a few years. Light spots following the acute rash may persist for longer.
Peramiquel L, Baselga E, Dalmau J, et al: Lichen striatus: clinical and epidemiological review of 23 cases, Eur J Pediatr
165:267–269, 2006.
PHOTODERMATOLOGY
121. Why is limiting excessive sun exposure in children important?
Many people experience a significant percentage of their lifetime sun exposure early in life. Years of unprotected sun exposure will lead to freckling, wrinkling, and skin cancer formation, including
melanoma. In an era of rising rates of melanoma and squamous and basal cell carcinomas, the use of sun-protection strategies during the pediatric years could lower the risk to an individual.
122. Why should indoor tanning be addressed and discouraged in children? Indoor tanning has become common among adolescent girls. In a recent study, 24% of high school girls reported indoor tanning use. Indoor tanning is associated with an increased risk of melanoma in adulthood. One study found that indoor tanning before the age of 35 years increases the risk of melanoma by 59%. Many, but not all, states now have legislation restricting tanning bed use among minors.
Guy GP Jr, Berkowitz Z, Tai E, et al: Indoor tanning among high school students in the United States, 2009 and 2011, JAMA Dermatol 150:501–511, 2014.
123. What are good strategies for protection against sun exposure?
• Seek shade when possible and remember that the sun’s rays are strongest between 10 AM and 2 PM.
• Remember that water, snow, and sand reflect the sun’s rays and may increase your chance of sunburn.
• Wear protective clothing, hats, and sunglasses.
• Apply sunscreen at least 30 minutes before sun exposure.
• Use a broad-spectrum sunscreen with an SPF of 30 or higher.
• Apply liberal amounts of sunscreen: about 1 oz (shot glass size or 30 mL) is enough to cover the exposed skin areas of an average sized adult; 15 mL is typically required for a 7-year-old child.
• Use a water-resistant sunscreen, and reapply sunscreen every 80 minutes during water-based activities or when sweating.
• Wear lip protection that contains sunscreen.
American Academy of Dermatology: www.aad.org/media-resources/stats-and-facts/prevention-and-care/sunscreens. Accessed on Mar. 14, 2015.
124. How is the SPF of a sunscreen determined?
SPF is the level of effectiveness of a sunscreen’s ability to protect against UVB light. It is not a measurement of UVA light protection. The SPF rating is a ratio of the dose of ultraviolet light needed to produce minimal redness on sun-protected skin to the dose of ultraviolet light needed to produce minimal redness on unprotected skin.
125. Should sunscreens be avoided in infants?
This is controversial. There are concerns that the skin of infants <6 months of age has different absorptive characteristics and that biologic systems that metabolize and excrete drugs may not be fully developed. Therefore, clothing protection and sun protective behaviors are advised for children
<6 months of age. Physical protection (e.g., clothing, hats, shade, sunglasses) is ideal, but if an infant’s skin is not adequately protected, it may be reasonable to apply sunscreen to small areas, such as the
face and the back of the hands. Physical sunscreens containing zinc oxide are preferred over chemical sunscreens for use on infant skin.
Committee on Environmental Health and Section on Dermatology: Ultraviolet light: a hazard to children and adolescents,
Pediatrics 127:588–597, 2011.
American Academy of Pediatrics: www.healthychildren.org/English/ages-stages/baby/bathing-skin-care/Pages/Baby- Sunburn-Prevention.aspx. Accessed on Mar. 14, 2015.
126. Do we risk developing vitamin D deficiency by using sun protection?
Vitamin D synthesis is a beneficial effect of exposure to ultraviolet light and several studies suggest an association between low vitamin D levels and the development of cancer. This however should not be interpreted as a reason to seek a tan. In most people, adequate amounts of vitamin D can be obtained by ingesting a healthy diet including foods that naturally contain or are fortified with vitamin D. The American Academy of Pediatrics has increased their recommended daily intake of vitamin D to prevent rickets and vitamin D deficiency in children.
Wagner Cl, Greer FR, AAP section on breastfeeding: Prevention of rickets and vitamin D deficiency in infants, children and adolescents, Pediatrics 122:1142–1152, 2008.
127. Which “lime” disease is not transmitted by ticks? Limes contain psoralens that react with ultraviolet light and that can produce erythema, vesicles, and/or hyperpigmentation on areas of the skin that have come in contact with lime juice. This is known as phytophotodermatitis and is seen with other psoralen-containing plants, such as celery and figs. Additionally, berloque dermatitis (berloque is French for “pendant,” which some lesions can resemble) is an irregularly patterned hyperpigmentation of the neck due to photosensitization by furocoumarins (i.e., psoralens) in perfumes. It is caused by fragrances that contain bergamot oil, an extract from the peel of a type of orange that is grown in southern France and Italy. Bergamot oil contains
5-methoxypsoralen, which enhances the erythematous and pigmentary response of UVA light.
128. Which conditions are associated with marked sun sensitivity?
• Inherited disorders: Porphyrias, xeroderma pigmentosum, Bloom syndrome, Rothmund- Thomson syndrome, Hartnup disorder
• Exogenous agents: Drugs (e.g., tetracyclines, thiazides), photoallergic contact dermatitis (associated with perfumes and para-aminobenzoic acid esters)
• Systemic disease: Lupus erythematosus, dermatomyositis
• Idiopathic disorders: Polymorphous light eruption, solar urticaria, actinic prurigo, hydroa vacciniforme
Chantorn R, Lim HW, Shwayder TA: Photosensitivity disorders in children: part I, J Am Acad Dermatol 67:1093.e1–e18, 2012. Chantorn R, Lim HW, Shwayder TA: Photosensitivity disorders in children: part II, J Am Acad Dermatol 67:1113.e1–e15, 2012.
129. What is the appearance of polymorphous light eruption? The most common pediatric photodermatosis, polymorphous light eruption is characterized by itchy red papules, plaques, or papulovesicles that appear several hours to days after ultraviolet light exposure. It can be diagnosed by phototesting (i.e., the induction of lesions by intentional ultraviolet light exposure) and by skin biopsy. It is usually suggested by the classic history and the exclusion of other photosensitivity disease.
Gruber-Wackernagel A, Byrne SN, Wolf P: Polymorphous light eruption: clinical aspects and pathogenesis, Dermatol Clin
32:315–334, 2014.
130. Is a child with sun sensitivity protected by sitting behind a window? Yes and no, depending on the reason for the sensitivity. Ultraviolet light is divided into three wavelength groups: ultraviolet C (UVC), 200 to 290 nm; UVB, 290 to 320 nm; and UVA, 320 to 400 nm. UVC light is cytotoxic and can cause retinal injury, but fortunately it is almost completely absorbed by the ozone layer. UVB light causes sunburn; dermatologic flares (e.g., in patients with lupus erythematosus); and, with chronic exposure, skin cancer. UVA light (which is also emitted from the fluorescent lamps used in most schools) is responsible for psoralen and drug phototoxicity and porphyria flares. It can cause skin
cancer with chronic exposure. Windows block UVB light, but not UVA. Thus, children with UVA-sensitive disorders are not protected by sitting behind a window unless it has been pretreated with a UVA filter.
PIGMENTATION DISORDERS
131. What disorders of childhood are associated with areas of hypopigmentation? Hypopigmentation is caused by a decrease—not a total absence—of pigmentation or melanin. Conditions that feature hypopigmented lesions include tuberous sclerosis, tinea versicolor, pityriasis alba, nevus depigmentosus, hypomelanosis of Ito, leprosy, and postinflammatory hypopigmentation.
132. Is treatment helpful for children with postinflammatory hypopigmentation?
In children with pityriasis alba, very-low-potency topical steroids, emolliation, and sun protection measures can make skin color more uniform. Treatment does not seem to help in other cases of postinflammatory hypopigmentation, such as those that occur after dermatitis, infection, abrasions, or burns, but sun protection is advisable.
133. What treatments are available for vitiligo?
Vitiligo is a disorder of depigmentation (total absence of pigmentation with sharp demarcations; Fig. 4-18). The etiology is unknown but may be autoimmune in nature. There are rare associations with other autoimmune conditions, including thyroiditis and juvenile-onset diabetes. Treatment may be unsatisfactory. Potent topical steroids have been used for localized areas.a Off-label topical tacrolimus ointmenta, b has been used with some success to treat facial vitiligo in children. Ultraviolet light therapy has been employed for some children with severe, extensive disease. More recently, excimer laser (308 nm) has been instituted as a potentially effective treatment for focal disease. Dyes (including self- tanning agents) and coverage cosmetics are often helpful for camouflaging skin lesions.
National Vitiligo Foundation: www.mynvfi.org. Accessed on Mar. 20, 2015.
Figure 4-18. Vitiligo. Note well-demarcated areas of total depigmentation. (From White GM, Cox NH: Diseases of the Skin: A Color Atlas and Text. London, 2002, Mosby, p 286.)
134. What conditions are associated with congenital depigmentation of the skin? Congenital depigmentation, or albinism, constitutes a number of genetically inherited syndromes that are characterized by disorders of melanin synthesis and that may affect the skin, hair, and eyes.
Generalized (oculocutaneous) albinism is often complicated by ocular abnormalities, including visual impairment, photophobia, and nystagmus. Piebaldism is a distinct form of congenital depigmentation that affects segments of skin. Patients with this condition often have a forelock of white hair, which is caused by a genetic mutation that differs from generalized albinism. Localized congenital depigmentation associated with a white forelock, heterochromia irides, and congenital deafness characterizes Waardenburg syndrome.
135. What is the likely diagnosis if a patient taking trimethoprim–sulfamethoxazole develops a single erythematous, sharply marginated, round lesion that leaves an area of hyperpigmentation upon resolution?
Fixed drug eruption. These 2- to 10-cm red to violaceous inflammatory plaques, are usually solitary and may blister. This hypersensitivity reaction occurs after medication ingestion (commonly antibiotics), especially trimethoprim–sulfamethoxazole and tetracycline. The resultant hyperpigmentation helps make the distinction.
Morelli JG, Tay YK, Rogers M, et al: Fixed drug eruptions in children, J Pediatr 134:365–367, 1999.
136. Why are Spitz nevi and malignant melanoma often confused? The Spitz nevus can appear suddenly and grow rapidly. Histologically, it has many features that can be mistaken for malignancy. It actually was previously referred to as benign juvenile melanoma. “Benign” is the key word for this red to brown, dome-shaped papule, which usually appears on the face or extremity. Clinicopathologic correlation is the key to making this diagnosis. It is essential that an experienced pathologist interpret the biopsy when a Spitz nevus is suspected. Melanoma in childhood has been misdiagnosed as Spitz nevi, and Spitz nevi have been misdiagnosed as melanoma.
Murphy ME, Boyer JD, Stashower ME, et al: The surgical management of Spitz nevi, Dermatol Surg 28:1065–1069, 2002.
137. In children with pigmented nevi, what factors increase the risk of melanoma? Melanoma is rare during childhood. If there is a family history of melanoma or atypical moles, a history of severe sunburns before the age of 18 years, or the child has a giant congenital nevus, the risk is greater. The risk increases with age and in those with fair skin (Fitzpatrick skin type), those children who tan poorly and freckle. Estimated risks vary for different-sized congenital nevi. The projected lifetime risk for a melanoma developing within a congenital nevus is controversial. For small congenital nevi, the risk is low. For giant congenital nevi, the risk is estimated to be 6% to 8%. Acquired nevi very rarely develop melanomas.
Gibbs NF, Makkar HS: Disorders of hyperpigmentation and melanocytes. In Eichenfield LF, Frieden IJ, Esterly NB, editors:
Textbook of Neonatal Dermatology, ed 2. Philadelphia, 2008, WB Saunders, pp 397–421.
138. How do the ABC’s of pediatric melanoma differ from adult melanoma? The conventional melanoma detection criteria in adults are based on the “ABCDE” rules, which stand for Asymmetry, Border irregularity, Color variegation, Diameter >6 mm, and Evolution. In a recent retrospective review of pediatric melanoma, Amelanosis, Bleeding, “Bumps,” uniform Color, variable Diameter, and De novo development were also proposed additional descriptors for detecting melanoma
in the pediatric population.
Cordoro KM, Gupta D, Frieden IJ, et al: Pediatric melanoma: results of a large cohort study and proposal for modified ABCD detection criteria for children, J Am Acad Dermatol 68:913–925, 2013.
VASCULAR BIRTHMARKS
139. How are vascular birthmarks classified?
The updated biologic classification of vascular birthmarks is the most widely accepted classification of vascular birthmarks. It was first proposed in 1982 and was adapted recently to reflect new knowledge. Two broad categories of vascular birthmarks are described: vascular tumors and vascular malformations. There are many types of vascular tumors, but infantile hemangiomas are the most
common. These demonstrate cellular hyperplasia. Vascular malformations, which are composed of dysplastic, malformed vessels, are categorized on the basis of their flow characteristics and type of anomalous channels. The majority of vascular anomalies seen in childhood fit into these categories. Vascular tumors (selected):
• Infantile hemangioma
• Congenital hemangioma
• Kaposiform hemangioendothelioma
• Tufted angioma
• Pyogenic granuloma
Vascular malformations:
• Capillary malformation (port wine stains, salmon patch)
• Lymphatic malformation (lymphangioma microcystic, macrocystic)
• Venous malformations
• Arteriovenous malformations
• Combined malformations
Wassef M and the Scientific Committee of the International Society for the Study of Vascular Anomalies: Updated ISSVA classification. Melbourne, Australia, April 2014.
Mulliken JB, Glowacki J: Hemangiomas and vascular malformations in infants and children, Plast Reconstr Surg
69:412–420, 1982.
140. What is the natural history of untreated infantile hemangiomas? Hemangiomas or, more specifically, infantile hemangiomas, are common benign vascular tumors. They are rarely fully developed at birth, but precursor lesions (an area of pallor, telangiectasia, or “bruise”) may be detected on close inspection within the first few days of life. They may have superficial and/or deep components. Hemangiomas undergo a growth phase until the child reaches the age of 6 to
12 months, at which time the tumors start to involute. Hemangiomas mark out their territory early and thereafter may grow in thickness or volume. Growth is nonlinear and often most dramatic in the first few months of life with many reaching 80% of their final size by 3 months of age. This process of involution occurs over several years. There still may be residual skin changes (e.g., skin redundancy, pallor, atrophy, telangiectasia) after the hemangioma has resolved. Management of infantile hemangiomas needs to be individualized because many factors are assessed in the decision to actively treat an infantile hemangioma.
Luu M, Frieden IJ: Haemangioma: clinical course, complications and management, Br J Dermatol 169:20–30, 2013.
141. What are the major goals of the management of infantile hemangiomas? The decisions regarding which hemangiomas require treatment and the best therapeutic modalities may not always be easy ones. The major goals of management are as follows:
• Prevent or reverse life- or function-threatening complications.
• Treat ulcerated hemangiomas.
• Prevent permanent disfigurement caused by a rapidly enlarging lesion.
• Minimize psychosocial stress for the family and the patient.
• Avoid overly aggressive procedures that may result in scarring in lesions that have a good likelihood of involuting without significant residual lesions.
Luu M, Frieden IJ: Haemangioma: clinical course, complications and management, Br J Dermatol 169:20–30, 2013.
142. What can patterns of hemangiomas on the skin surface tell us?
The characteristics of hemangiomas on the skin surface can provide clues to underlying abnormalities and help predict outcomes. Hemangiomas are described as being superficial when they are located in the upper dermis and present as red papules and plaques (“strawberry”). Deep hemangiomas are located in the deeper dermis and subcutaneous tissue and are often blue-purple in color. Mixed hemangiomas have both superficial and deep components (“iceberg phenomenon”). Hemangiomas are also characterized based on their distribution on the skin surface. Hemangiomas that appear to arise from a single focus are called “localized” or “focal” hemangiomas, and lesions that cover a broad area on the skin surface, which might represent a developmental subunit, are called “segmental.”
Multiple focal lesions (multifocal) may herald internal hemangiomas, and segmental hemangiomas may predict involvement with other anatomic and developmental disorders (PHACE syndrome).
Haggstrom AN, Lammer EJ, Schneider RA, et al: Patterns of infantile hemangiomas: new clues to hemangioma pathogenesis and embryonic facial development, Pediatrics 117:698–703, 2006.
143. Which hemangiomas are especially worrisome?
• Multiple cutaneous hemangiomas may be associated with visceral hemangiomas (most commonly liver hemangiomas).
• Large/bulky hemangiomas may cause significant disfigurement of underlying structures.
• Segmental hemangiomas are associated with PHACE and LUMBAR syndromes.
• “Beard” hemangiomas may be a marker for underlying laryngeal or subglottic hemangiomas that may impair respiratory function.
• Midline spinal hemangiomas may be a marker for an underlying spinal cord abnormality.
• Head and neck hemangiomas, usually segmental lesions >5 cm in diameter, may be associated with other congenital anomalies, including central nervous system, cardiac, ocular, and sternal defects (e.g., posterior fossa malformation, hemangioma, arterial abnormalities, coarctation, eye
abnormalities, sternal defects [PHACE(S)] syndrome).
• Vulnerable anatomic locations impair vital functions and cause disfigurement (e.g. periocular, neck, lip, nasal tip).
• Ulcerated hemangiomas increase risk of superinfection, can bleed, cause pain, and lead to scarring.
Haggstrom AN, Drolet BA, Baselga E, et al: Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment, Pediatrics 118:882–887, 2006.
144. When is treatment indicated for infantile hemangiomas?
• Lesions that interfere with normal physiologic functioning (i.e., breathing, hearing, eating, vision), especially periocular hemangiomas (to prevent amblyopia)
• Recurrent bleeding, ulceration, or infection
• A rapidly growing lesion that distorts facial features or has the potential to result in a residual lesion that will cause disfigurement
Luu M, Frieden IJ: Haemangioma: clinical course, complications and management, Br J Dermatol 169:20–30, 2013.
145. What are the most commonly used treatments for problematic infantile hemangiomas?
In most cases, topical treatments are used for smaller superficial lesions that do not have a significant deep component; systemic medications are used for larger deeper infantile hemangiomas, those with an aggressive growth pattern, or those that require more aggressive therapy. Until recently, there were no FDA-approved treatments for infantile hemangioma. In March of 2014, a specific formulation of oral
propranololb received FDA approval for treatment of infants >5 weeks with problematic infantile hemangiomas. Other medications used for hemangioma remain “off label.”
Beta blockers (topical timolol–gel forming solutiona and oral propranolola): Topical timolol– gel forming solution, an ophthalmologic preparation, has been reported to be effective for the treatment of superficial hemangiomas. Oral propranolol was first reported to be efficacious for halting the proliferation and speeding the regression of infantile hemangiomas in 2008. In many cases, it has become the first-line therapy for problematic hemangiomas.
Corticosteroidsa (oral intralesional and topical): These were the mainstay of treatment for hemangiomas until several years ago. Their use has been largely supplanted by beta blockers.
Pulsed dye laser: This may be used with other modalities but is usually of little benefit in situations when systemic treatment with propranolol is indicated. Limited penetration restricts use to superficial lesions. It can be useful for painful ulcerated hemangiomas that fail to respond to other treatment modalities.
Surgery: Surgical excision is most commonly used to manage the residual lesions of hemangiomas. Facial lesions that leave a significant residual lesion are often managed around age 3 years before the child starts preschool.
Luu M, Frieden IJ: Haemangioma: clinical course, complications and management, Br J Dermatol 169:20–30, 2013.
KEY POINTS: WORRISOME HEMANGIOMAS
• Multiple hemangiomas: May be associated with visceral hemangiomas (most commonly liver hemangiomas)
• Large/bulky hemangiomas: May cause significant disfigurement of underlying structures
• Segmental hemangiomas: Associated with PHACE and LUMBAR syndromes
• “Beard” hemangiomas: May be a marker for underlying laryngeal or subglottic hemangioma that may impair respiratory function
• Midline spinal hemangiomas: May be a marker for underlying spinal cord abnormality
• Head and neck hemangiomas: Usually segmental lesions> 5 cm in diameter, may be associated with other congenital anomalies
• Vulnerable anatomic locations: Impair vital functions or cause disfigurement (e.g., periocular, neck, lip, nasal tip)
• Ulcerated hemangiomas: Increased risk of infection, cause pain and lead to scarring
146. Why is an infant with a vascular tumor and new-onset thrombocytopenia so worrisome?
This can indicate the development of the Kasabach-Merritt syndrome (or phenomenon), a life-threatening condition of rapidly enlarging vascular tumors and progressive coagulopathy.
Platelets are sequestered within the lesion(s), forming thrombi and consuming coagulation factors. Ecchymoses may develop initially around the vascular tumor, but a disseminated coagulopathy with anemia can result. Aggressive therapy (often systemic steroids and/or vincristine, and surgery) is frequently needed. Kasabach-Merritt syndrome is not caused by common infantile hemangiomas but rather by two rare vascular tumors (Kaposiform hemangioendothelioma
and tufted angioma).
147. How do superficial hemangiomas differ from port wine stains?
Superficial hemangiomas are superficial, palpable, vascular tumors that usually involute with time. In the past, they were called “strawberry” hemangiomas. A port wine stain, sometimes called nevus flammeus, is a type of capillary malformation that results in flat vascular malformations composed of capillary and postcapillary venule-sized vessels that do not involute. Some superficial hemangiomas may mimic port wine stains during the first few
weeks of life; observation of their growth pattern is helpful for establishing the correct diagnosis (Table 4-5).
Table 4-5. Superficial Hemangiomas Versus Port Wine Stains
SUPERFICIAL HEMANGIOMAS PORT WINE STAINS
Palpable Flat, macular
Common (4–10% of children <1 year old) Less common (0.1–0.3%)
Often not apparent at birth (more visible at 2–12 weeks) Present at birth
Bright red Pale pink to blue-red (darkens with age)
Well-defined borders Borders variable
Pathology: Proliferating angioblastic endothelial cells with variable blood-filled capillaries Pathology: dermal capillary dilation
Majority involute spontaneously by age 9 years No involution: may worsen with darkening and hypertrophy
Rapid growth phase Proportionate growth (as child grows)
Suggested therapy: watchful waiting; active treatment for some lesions Suggested therapy: Flash lamp pulsed- dye laser often used for facial lesions
148. What is “simple” about a nevus simplex?
NEVUS Simplex, also known as “stork bites” when they are on the nape of the neck and “angel’s kiss” when located in the glabella and eyelids, are very common. Pink to red in color, they often fade without treatment in the first few years of life. Thus, reassurance and monitoring is usually the only treatment recommended, which is a “simple” solution. Their characteristic location helps differentiate them from true facial port wine stains, which do not fade with time and may
require treatment.
149. When are port wine stains associated with other anomalies?
Sturge-Weber syndrome (Fig. 4-19) refers to the association of a facial port wine stain (typically affecting the skin innervated by the first branch of the trigeminal nerve), an ocular vascular anomaly linked with glaucoma, and a leptomeningeal vascular anomaly associated with seizures and developmental delay.
Klippel-Trenaunay syndrome refers to the association of a limb port wine stain (usually lower extremity) with ipsilateral soft tissue and bony overgrowth and venous/lymphatic anomalies.
Figure 4-19. Sturge-Weber syndrome. The bilateral port wine stain involves the V1, V2, and V3 regions and the right V3. (From Sahn EE: Dermatology Pearls. Philadelphia, 1999, Hanley & Belfus, p 225.)
150. How are port wine stain–type capillary malformations treated? Port wine stains are often pink to dark red in color during childhood. With maturity they often darken and take on their “port wine” color. Treatment of facial capillary malformations is generally recommended during infancy or early childhood when the lesions appear to be more amenable to therapy with the pulsed dye laser. The pulsed dye lasers that are used for treatment of port wine stains are designed to target oxyhemoglobin and lead to destruction of the blood vessels and subsequent lightening of the stain. Multiple treatments sometimes requiring general anesthesia or sedation are required to achieve lightening. It is important for patients and families to understand that this treatment often achieves cosmetically acceptable lightening but complete disappearance/removal of the birthmark is not yet possible with the current technologies. In some patients the stain will redarken after treatment, and touch-up treatments may be required.
VESICOBULLOUS DISORDERS
151. What is the Nikolsky sign?
This sign demonstrates epidermal fragility. Gentle lateral pressure placed on apparently intact skin causes an erosion, especially near preformed vesicles. This sign is positive in several autoimmune, infectious, and inherited blistering conditions, such as bullous pemphigoid, staphylococcal scalded skin syndrome (SSSS), and epidermolysis bullosa.
152. What are causes of skin blistering in childhood?
• Infectious: Bacterial (bullous impetigo, SSSS), viral (herpes simplex virus, varicella)
• Contact dermatitis: Poison ivy, phytophotodermatitis
• Inherited disorders: Epidermolysis bullosa, bullous congenital ichthyosiform erythroderma
• Autoimmune disorders: Linear immunoglobulin A disease, bullous pemphigoid, pemphigus vulgaris
• Other: Erythema multiforme, toxic epidermal necrolysis, thermal injury (burns)
153. How is SSSS differentiated from toxic epidermal necrolysis (TEN)? Both are diffuse bullous diseases. SSSS commonly arises in young children <5 years old and develops after a localized staphylococcal infection with diffuse cutaneous disease caused by an exfoliative toxin. The level of blistering includes the superficial levels of the epidermis. TEN is believed to be a hypersensitivity reaction (often to a drug) and occurs in all age groups. The level of blistering in TEN is
deep, and the entire epidermis is necrotic. (Table 4-6).
Table 4-6. Differentiation Between Staphylococcal Scalded Skin Syndrome and Toxic Epidermal Necrolysis
CHARACTERISTICS STAPHYLOCOCCAL SCALDED SKIN SYNDROME
TOXIC EPIDERMAL NECROLYSIS
Etiology Infectious; group II staphylococci Immunologic; usually drug related
Morbidity/mortality Low with treatment High
Mucous membrane involvement Rare Frequent
Nikolsky sign Present Absent
Target lesions Absent Often present
Level of blister Upper epidermis (below stratum corneum) Subepidermal
Histopathology No epidermal necrosis or dermal inflammation Full-thickness epidermal necrosis; prominent perivascular dermal inflammation
Adapted from Roberts LJ: Dermatologic diseases. In McMillan JA, DeAngelis CD, Feigin RD, et al editors: Oski’s Pediatrics, Principles and Practice, ed 3. Philadelphia, 1999, Lippincott Williams & Wilkins, p 379.
154. Why are neonates susceptible to SSSS? The answer lies in the fact that newborns share their susceptibility to SSSS with patients in renal failure. It is the reduced clearance of the exfoliative toxin by the newborn’s immature kidneys that contributes to their increased susceptibility to SSSS.
155. Where can the S. aureus be found in patients with SSSS?
S. aureus commonly colonizes the nasopharynx and the umbilicus. The source of infection may also be present in the urinary tract, a wound, conjunctiva, or blood. The bacteria are not usually present at the site of the skin lesions because they are the result of a systemic toxin-mediated effect.
156. What is the likely diagnosis for a 4-year-old child who develops a 1-week history of widespread painful and pruritic bullous lesions with crusted lesions around which vesicles are arranged in a string-of-pearls appearance?
Chronic bullous dermatosis of childhood. This is the most common acquired autoimmune bullous disease of young children. It is characterized on biopsy by immunoglobulin A (IgA) and C3 deposition along the basement membrane (sometimes called linear IgA bullous dermatosis). Although the differential diagnosis of bullous diseases is large, the appearance of new vesicles or bullae in a string of pearls (or cluster-of-jewels) appearance around crusted or erythematous plaques is characteristic.
Sheehan M, Huddleston H, Mousdicas N: Chronic bullous dermatosis of childhood, Arch Pediatr Adolesc Med
162:581–582, 2008.
157. What are the subtypes of epidermolysis bullosa (EB)?
EB is a heterogeneous group of inherited disorders characterized by the formation of blisters and erosions at sites of friction or trauma. There are three subtypes of EB that are categorized based on the region of the dermal-epidermal junction (DEJ) that they affect: simplex, junctional, and dystrophic. The extent of blistering and the degree of scarring roughly correlate with the level of blister formation in the epidermis or dermis. A fourth subtype of EB has been added, Kindler syndrome, which affects multiple layers of the DEJ.
Dystrophic Epidermolysis Bullosa Research Association of America: www.debra.org. Accessed on Mar. 23, 2015.
158. Should epidermolysis bullosa blisters be “popped”? Yes. Because patients with EB have a genetic abnormality of the proteins that hold their epidermis and dermis together, the pressure from simple accumulation of fluid within an intact blister can cause the blister to expand. The blister should be drained by a sterile needle or lancet after a gentle sterile alcohol prep of the site. The blister roof should be left intact.
159. Which disorder is associated with “target lesions”?
Erythema multiforme (EM). This is typically an acute, self-limited, but sometimes recurring skin condition (EM minor) believed to be a T cell–mediated immune reaction most commonly to certain infections (e.g., herpes simplex virus, streptococcal, Epstein-Barr virus [EBV]) but also to a variety of other triggers, particularly medications (e.g., sulfa drugs, penicillins, anticonvulsants). In EM major, erosions or bullae of the oral, genital, or ocular mucosa occur. Most mild cases resolve over 1 to
2 weeks. The characteristic target lesions occur as dusky, red papules that evolve into depressed, localized, damaged epithelium with spreading annular edema (pale) and inflammation on the periphery (erythema) (Fig. 4-20).
Figure 4-20. Erythema multiforme with typical target lesions. (From Goldbloom RB: Pediatric Clinical Skills, ed 4. Philadelphia, 2011, ELSEVIER Saunders, Color plate 19-11.)
160. Is EM part of a continuum with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)?
This is an area of controversy. Traditionally, these had been viewed as related disorders because they had some shared clinical and histologic features. However, the majority of EM cases are associated with infections, while SJS/TEN occurrences are believed to be drug-related in >90% of cases. There are also distinctions according to anamnesis (history), morphology, involved sites, extension of lesions, and
pathogenic mechanisms. Current opinion favors these as distinct conditions, with SJS/TEN not being the end-stage result of some cases of EM.
Iwai S, Sueki H, Watanabe H, et al: Distinguishing between erythema multiforme major and Stevens-Johnson syndrome/ toxic epidermal necrolysis immunopathologically, J Dermatol 39:781–786, 2012.
Roujeau JC: Stevens-Johnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme, J Dermatol 24: 726–729, 1997.
161. What distinguishes SJS from TEN?
Both are severe mucocutaneous disorders that are characterized by extensive necrosis and epidermal detachment. They are believed to be a continuum, usually triggered by medications, and are distinguished primarily based on the percentage of body surface that is involved. SJS is the less severe of the two, defined as <10% body surface involvement. TEN encompasses >30% of body area.
Between 10% and 30% indicates a classification overlap.
162. What medications are most commonly associated with SJS and TEN in children? Sulfonamides and anticonvulsants, especially phenobarbital, carbamazepine, and lamotrigine. Less common causes are penicillins and NSAIDs. Very rarely, paracetamol (acetaminophen) may be involved.
Ferrandiz-Pulido C, Garcia-Patos V: A review of causes of Stevens-Johnson syndrome and toxic epidermal necrolysis in children, Arch Dis Child 98: 998–1003, 2013.
163. Is steroid therapya beneficial for the treatment of SJS or TEN? This is a continuing area of controversy; studies are inconclusive. In SJS, treatment may be considered early during the course if multiple mucosal surfaces are involved, but skin denudation is limited. The potential for steroidsa to increase medical complications (e.g., hemorrhage, infection) must be taken into account, but a large 2008 study found that it was lower than previously thought. If initiated, clinical response (or lack thereof) should be carefully followed, and steroids should be discontinued if the condition is worsening. Because the majority of cases of SJS will spontaneously resolve, other therapies are vital: skin care, nutritional support, ophthalmologic care, and the treatment of secondary bacterial infections. Steroids have been reported to be associated with an increased mortality rate among patients with TEN.
Koh MJ-A, Yay Y-K: An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children, Curr Opin Pediatr 21:505–510, 2009.
Schneck J, Fagot JP, Sekula P, et al: Effects of treatment on the mortality of Stevens-Johnson syndrome and toxic epidermal necrolysis: a retrospective study on patients included in the EuroSCAR study, J Am Acad Dermatol 58:33–40, 2008.
164. What therapy should be considered for patients with rapidly progressive SJSor TEN? Intravenous immune globulina should be considered. Caution should be used, especially in patients with poor renal function, hypercoagulable states, or IgA deficiency.
Momin SB: Review of intravenous immunoglobulin in the treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis, J Clin Aesthet Dermatol 2:51–58, 2009.
165. What infection should be considered in a child with SJS and a cough?
Mycoplasma pneumonia induces mucosal erosions that mimic those of drug-induced SJS.
Bullen LK, Zenel JA: A 15-year-old female who has cough, rash and painful swallow, Pediatr REV 26:176–181, 2005.
166. In addition to hand-foot-mouth, which other body site is often affected in children with coxsackie virus infections?
Coxsackie viral infections often also cause skin lesions on the buttocks, prompting some clinicians to call the condition “hand-foot-mouth-butt syndrome.”
Mohr MR, Sholtzow M, Bevan HE, et al: Exploring the differential diagnosis of hemorrhagic vesicopustules in a newborn,
Pediatrics 127:e226–e230, 2011.
Acknowledgment
The editors gratefully acknowledge contributions by Dr. Robert Hayman, Dr. Leonard Kristal, and Dr. Vivian Lombillo that were retained from prior editions of Pediatric Secrets.
BONUS QUESTIONS
167. Can acne be scrubbed away?
No. In fact, scrubbing can often irritate the skin, further contributing to inflammation.
168. Can lasers be used to treat acne?
Yes. Select light-based lasers are an alternative option to treat acne. Multiple treatments and touch-ups are typically required. Residual acne scarring can also be treated with lasers such as a pulsed
dye laser to hasten lightening of pink or reddish scars and ablative lasers to improve atrophic or pitted scars.
169. Are there any “quick fixes” for acne? Individual inflamed acne cysts may be treated with intralesional injection of a low-potency corticosteroid. Side effects may include skin atrophy. This option should not be used for patients with numerous lesions because systemic absorption may occur, especially when larger amounts of the medication are used.
170. Can dilute bleach be used for purposes other than cleaning your clothes? Historically, dilute bleach (sodium hypochlorite) has been used to decrease bacteria on the skin of patients with burn wounds. The preparation must be diluted further than Dakin’s solution (0.5%) so that it will not harm the skin cells needed for wound healing but will still decrease bacterial colonization. Studies have been performed using dilute sodium hypochlorite baths for children with atopic dermatitis.
Ryan C, Shaw RE, Cockerell CJ, et al: Novel sodium hypochlorite cleanser shows clinical response and excellent acceptability in the treatment of atopic dermatitis, Pediatr Dermatol 30:308–315, 2013.
Huang JT, Abrams M, Tlougan B, et al: Treatment of Staphylococcus aureus colonization in atopic dermatitis decreases disease severity, Pediatrics 123:e808–e814, 2009.
171. Do soaps or clothes make any difference in atopic dermatitis? Yes. Mild soaps and soap-free cleansers designed for sensitive skin are better than drying or fragranced soaps. “Bubble baths” should be avoided, and cleansers should be applied at the end of the bath. Woolen fibers can also irritate the skin and trigger the itch-scratch cycle. Soft fibers, such as cotton, are the least irritating.
172. Frequency of bathing for children with atopic dermatitis: more or less? Recommendations on this topic vary. However, a 2014 study found no differences between a group that bathed daily for 7 days compared with another group that bathed only twice weekly. Adequate skin hydration with moisturizers was believed to be more important than a particular bathing schedule.
Koutroulis I, Petrova K, Kratimenos P, et al: Frequency of bathing in the management of atopic dermatitis: to bathe or not to bathe? Clin Pediatr 53:677–681, 2014.
173. What is the most common side effect of topical pimecrolimus and tacrolimus? These topical immunomodulating creams or ointments are used for intermittent therapy. The most common side effect is burning or stinging, which can occur in up to 10% of patients, especially with initial use. This side effect tends to improve with continued use. Use on open skin sores should be avoided.
Eichenfield LF, Hanifin JM, Luger TA, et al: Consensus conference on pediatric atopic dermatitis, J Am Acad Dermatol
49:1088–1095, 2003.
174. What is a cause of a localized triangular area of alopecia on the scalp of children? Temporal triangular alopecia (TTA), also called congenital triangular alopecia, is an uncommon but benign cause of a localized area of alopecia on the temporal scalp. In most cases, it is noted between the ages of 2 and 6 years and classically is nonprogressive; this helps to differentiate it from alopecia areata and trichotillomania, the two conditions for which it is most frequently mistaken.
Lee KC, Chamlin S: A 14-year-old girl with a patch of hairloss, Pediatr Ann 38:646–649, 2009.
175. What is the “flag sign”?
The term flag sign refers to alternating bands of decreased pigment or structural changes of the hair shaft. It most commonly occurs after nutritional deficiency.
Wade MS, Sinclair RD: Disorders of hair in infants and children other than alopecia, Clin Dermatol 20:16–28, 2002.
176. Is there an oral treatment available to treat scabies?
Yes. Off-label use of IVERMECTIn has been reported to treat scabies. It is often considered for patients with a significant dermatitis that precludes the application of topical permethrin. Ivermectin is not recommended for children <5 years of age or <15 kg because it may cross the immature blood-brain barrier.
177. What types of sunscreens are available?
Physical sunscreens are composed of zinc oxide or titanium dioxide and function by scattering ultraviolet light. Although they are opaque, newer micronized preparations are easier to apply and more acceptable to patients. Chemical sunscreens absorb either UVA or UVB light. Most commercially available sunscreens are combinations of various agents. For sunscreens to function well, they must be applied on all exposed surfaces in adequate quantities and reapplied throughout the day.
178. Describe causes of pseudoporphyria in children
Pseudoporphyria is a phototoxic vesicobullous eruption. It can occur with chronic nonsteroidal anti- inflammatory drug (NSAID) use (especially naproxen sodium) and is therefore not uncommon in patients with juvenile idiopathic arthritis. Other medications associated with pseudoporphyria include tetracyclines, dapsone, furosemide, nalidixic acid, retinoids, amiodarone, and voriconazole. It has also been reported in children receiving hemodialysis.
Paller AS, Mancini AJ: Photosensitivity and photoreactions. In Paller AS, Mancini AJ, editors: Hurwitz Clinical Pediatric Dermatology, ed 3. Philadelphia, 2006, Elsevier, p 520.
179. What are the clinical features of familial dysplastic nevus syndrome? Atypical or dysplastic NEVI share some features with melanoma; they range from 5 to 15 mm in diameter and are round to oval in shape. Furthermore, they have irregular and indistinct margins, exhibit variation in color within the same lesion, and have both macular and elevated components. They tend to occur in sun-protected areas. The syndrome, which is also known as the Familial Atypical Multiple
Mole-Melanoma (FAMMM) syndrome, is found in families who have acquired individual and/or conferred nevi that have an increased risk of developing into melanoma. In FAMMM, not only is there an increased risk of melanoma, but also extracutaneous malignancies as well.
180. Which hemangioma is an exception to the rule of gradual resolution?
Infantile hemangioma with minimal or arrested growth pattern (IH-MAG) is an infantile hemangioma that exhibits an exception to the usual growth pattern of hemangioma. Their appearance is characterized by a patch of coarse surface telangiectasias with a rim of pallor resembling a hemangioma precursor lesion. By definition, less than 25% of its surface will show proliferation. Other names
that have been used to describe an IH-MAG are reticular infantile hemangioma, abortive hemangioma, and minimal growth hemangiomas. It is important to recognize this pattern because infantile hemangiomas are sometimes mistaken for capillary malformations.
Suh KY, Frieden IJ: Infantile hemangiomas with minimal or arrested growth: a retrospective case series, Arch Dermatol
146:971–976, 2010.
181. What is “eczema coxsackium”? During seasonal enteroviral outbreaks, patients with atopic dermatitis may present in a dramatic fashion with widespread blistering, usually symmetrical with clusters around the mouth and acral areas more than truncal lesions. The condition may mimic bullous impetigo, eczema herpeticum, vasculitis, and primary immunobullous disease.
Mathes EF, Oza V, Frieden IJ, et al: “Eczema coxsackium” and unusual cutaneous findings in an enterovirus outbreak,
Pediatrics 132:e149–e157, 2013.