PEDIATRIC DERMATOLOGY

OFFICE DERMATOLOGY, PART I1

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PEDIATRIC DERMATOLOGY Sonali G. Hanson, MD, and James F. Nigro, MD

Pediatric dermatology may pose a challenge to pediatricians and dermatologists alike, because pediatricians may be unfamiliar with certain dermatologic diagnoses, and general dermatologists may not be accustomed to dealing with children and their parents. When making dermatologic diagnoses, it is important to examine the skin carefully, paying special attention to distribution of lesions; morphology; and hair, nail, and mucosal changes. Possible differential diagnoses also need to be kept in mind. For physicians who do not have routine experience with pediatric conditions, it may be difficult to adjust the patient-physician interaction to the appropriate level. For infants, obviously the majority of physician interaction occurs with parents or other caretakers. With increasing age of the patient, the physician should focus the visit more toward the patient. Patients should also be allowed to provide more input into treatment decisions as they become older. These factors are especially important when evaluating and managing teenage patients because compliance may become a major issue. Some more specific points need to be kept in mind when evaluating and managing cutaneous disease in children. Skin biopsies are usually a last resort because they may be traumatic for young children and leave unnecessary scarring. If the diagnosis is truly in question and the information provided by a biopsy would alter management of the patient, then it is imperative to perform this diagnostic test. For children who fear needles, the eutectic mixture of lidocaine and prilocaine (EMLA) may be applied to anesthetize the biopsy site before more definitive anesthesia with infiltration of lidocaine. When performing

From the Departments of Dermatology (SGH, JFN) and Pediatrics (JFN), Baylor College of Medicine, Houston, Texas

MEDICAL CLINICS OF NORTH AMERICA VOLUME 82 * NUMBER 6 * NOVEMBER 1998

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biopsies or any other procedures in a child, distraction is an important tool in allaying fear. At the authors’ hospital, a Child Life service is specifically devoted to providing distraction and comfort to children during procedures, by using books, games, music, and toys. Therapeutic options are weighed according to the age and maturity of each individual child. For a disease that is benign and self-limited, the physician, parent, and child may opt for no therapy at all except for watchful waiting; the same disease in an adult might be treated more aggressively. Traumatizing a child unnecessarily should be avoided. A common example of such a situation is the treatment of warts, which is discussed in more detail later. Systemic medications should be prescribed in liquid formulations whenever possible, until the child is able to tolerate swallowing pills or tablets, usually by 8 years of age. If a medication is not available in a liquid formulation and there is no other choice for treatment, tablets may be crushed and mixed with food or drink, and capsules may be opened for mixing as long as appropriate dosing can be maintained. Topical medications are used frequently in the treatment of dermatologic disease. Topical corticosteroids comprise a large portion of the medications prescribed in dermatology on a daily basis. They are classified according to potency, and different vehicles may change the potency of a particular steroid. Ointments are generally more potent than the same medications in cream preparations. Topical steroids used by the authors are listed according to potency ranking in Table 1. Physicians and patients must be vigilant for side effects of long-term topical steroid use, especially when using the more potent formulations. Adverse effects include atrophy of the skin, telangiectasia, and striae. Only class VII topical steroids should be used for treatment of inflammatory conditions on the thin skin of the face, underarms, and groin. Topical antibiotics are often used in the treatment of infectious dermatologic conditions. Specific topical medications that should be avoided in children are neomycin because of a high risk of contact sensitization, topical lidocaine, and topical diphenhydramine. Another important factor in management of the pediatric patient is education about the disease process, both for the parents and for the mature child. For example, in chronic diseases such as atopic dermatitis and acne, it is important for the parents and the patient to understand the chronic nature of the disease, the importance of long-term management, and the need for acute treatment when appropriate. In the management of benign skin lesions such as warts, it is important for the parents and child to understand the cause and self-limited nature of the infection, whether treated or not. Education enhances rapport and increases compliance. This article discusses specific common dermatologic entities within the broad categories of infectious disease, papulosquamous and eczematous disorders, inflammatory disease, and neoplastic disease. To begin, diagnostic techniques used in pediatric dermatology are discussed.

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Table 1. POTENCY RANKING OF COMMONLY USED TOPICAL STEROIDS

Group 1 (Most Potent) Clobetasol propionate ointment 0.05% (Temovate, Cormax) Clobetasol propionate cream 0.05% (Temovate, Cormax) Betamethasone dipropionate ointment 0.05% (Diprolene) Group 2 Betamethasone dipropionate cream 0.05% (Diprolene) Mornetasone furoate ointment 0.1% (Elocon) Fluocinonide ointment 0.05% (Lidex) Fluocinonide gel 0.05% (Lidex) Fluocinonide cream 0.05% (Lidex) Group 3 Fluticasone propionate 0.005% (Cutivate) Group 4 Mometasone furoate cream 0.1% (Elocon) Triamcinolone acetonide ointment 0.1Yo (Kenalog) Hydrocortisone valerate ointment 0.2% (Westcort) Group 5 Fluticasone propionate cream 0.05% (Cutivate) Triamcinolone acetonide 0.1% (Kenalog) Hydrocortisone valerate cream 0.2% (Westcort) Group 6 Alclometasone dipropionate 0.05% ointment (Aclovate) Alclometasone dipropionate 0.05% cream (Aclovate) Desonide 0.05% ointment (DesOwen) Desonide 0.05% cream (DesOwen) Group 7 Topical preparations with hydrocortisone

DIAGNOSTIC TECHNIQUES USED IN PEDIATRIC DERMATOLOGY

In making dermatologic diagnoses, certain techniques are employed that can be useful in confirming clinical suspicions. One of the most basic techniques used in dermatology is the potassium hydroxide (KOH) preparation for the diagnosis of dermatophytosis. A No. 15 blade is used to scrape scale from a suspected tinea infection onto a glass slide. One to two drops of 10% to 20% KOH is then placed over the scale and covered with a coverslip. If available, a flame can be used to heat fix the preparation. Otherwise, waiting 10 to 15 minutes for the KOH to break apart cell walls effectively is also helpful. A survey of the slide should be made at low power, and when a suspected hypha is seen, higher power can be used for confirmation. It is sometimes difficult to distinguish hyphae from cell walls, and practice is needed to be able to confirm the presence of hyphae. In the case of equivocal KOH preparations, especially when considering the systemic treatment of tinea capitis or onychomycosis, a fungal

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culture can be sent for confirmation of diagnosis. The authors use a sterile moistened culturette stick to swab scale from the affected area for culture in the hospital laboratory. Tzanck smears may be helpful in the diagnosis of infections caused by the herpesvirus family. A sterile blade is used to unroof a vesicle, and the base is swabbed with a sterile cotton-tipped applicator. The material obtained from the base of the lesion is spread onto a glass microscope slide then gently heat fixed. After fixation, any blue or violet stain may be applied to the slide and left on for 1 minute. The authors prefer PMS or Giemsa. The stain is then rinsed off gently, and a coverslip is placed. The finding of multinucleated giant cells, or balloon cells, indicates the presence of herpes simplex, herpes zoster, or varicella virus, but the Tzanck smear cannot be used to distinguish among these three infections. In a patient with suspected scabies, a scabies preparation should be performed. A No. 15 blade is moistened with either mineral oil or water, and a burrow or papule is scraped vigorously to obtain upper epidermal material. The scrapings are spread onto a glass microscope slide then covered with one or two drops of mineral oil and a coverslip. Under low-power magnification, a search is made for the mite itself, ova, or feces. The mite is a six-legged arthropod, eggs are oval in shape, and feces are also oval but are significantly smaller than eggs and brown in color. Skin biopsy specimens provide valuable information in the appropriate setting. The intended biopsy site is infiltrated intradermally with a small amount of 1%lidocaine, usually with epinephrine, until a wheal is visible. The size of the biopsy specimen and whether a punch or shave biopsy is done depend on the morphology and differential diagnosis of the lesions in question. INFECTIOUS DISEASE Viral Infections of the Skin

The wart, or vermca, is one of the most common dermatologic diagnoses. There are several types of warts, including verruca vulgaris, or the common wart; verruca plana, or the flat wart; verruca plantaris, or plantar wart; and condylomata acuminata, or genital warts. Warts are caused by infection with the human papillomavirus (HPV). Many distinct types of HPV have been characterized, and some are more commonly associated with certain types of warts than others. For example, common warts are usually caused by infection with HPV types 1, 2, 4, or 7; flat warts are usually due to infection with HPV type 3; plantar warts are associated with HPV types 1 and 2; and genital warts are associated with HPV types 6, 11, 16, and 18. Verrucae vulgaris begin as pinpoint, skin-colored to whitish papules that may turn yellowish to gray as they enlarge. With time, the surface

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becomes more papillomatous, or verrucous. Verrucae plana are usually smooth, only slightly elevated skin-colored to tan papules. Plantar warts may become painful from the pressure of weight bearing. Genital warts are soft, flesh-colored to yellowish, usually smooth-topped but sometimes papillomatous papules. Genital warts can be sexually transmitted, and thus in young children the possibility of abuse must be considered. In children younger than 2 years of age who have had the warts since infancy and without any evidence of sexual abuse, sexual transmission may not be as great of a concern. Perinatal transmission is possible. In addition, innocent transmission of common warts to the genital area may occur via infected caretakers during activities such as bathing and changing of diapers. Warts may take months to years to resolve without treatment. Cosmetic issues, social stigma, and concern about transmission usually prompt the patient or parents to seek treatment. Before treatment, patients and parents should understand that if left alone, warts do not result in any harm to the patient. It should also be explained that there is no single effective treatment for warts, several treatments may be required before warts resolve, and the treated warts may recur or new warts may appear in different locations after treatment. Common treatment modalities rely on destruction of the infected tissue and include salicylic acid, cantharidin, trichloroacetic acid, and cryotherapy with liquid nitrogen. Cryotherapy, although effective, can be quite painful, and thus the authors do not generally use this treatment modality in children under the age of 8 years. Salicylic acid preparations are available over-the-counter and are the least painful method of treatment, although resolution may take much longer than with cryotherapy. If treatment with the above-mentioned modalities is not feasible, for example, in a young patient with many warts, oral cimetidine (Tagamet) may be considered. It has been reported to be useful in the treatment of warts,14 although other studies have shown that it is no better than placebo.2,*O Molluscum contagiosum is another common childhood viral infection of the skin. It is caused by a large DNA virus in the poxvirus group. As the name implies, the lesions are contagious and autoinoculable. Clinically, there are discrete skin-colored, dome-shaped umbilicated papules singly or in groups, which may have an erythematous base. They are usually asymptomatic. Principles of treatment are the same as discussed for warts because this infection is also benign and self-limited. These lesions, however, generally resolve more readily than warts. The treatment the authors prescribe most often, especially for multiple lesions, is topical tretinoin (Retin A). An option for treatment if there are few lesions is the expression of the core from the umbilicated center with either a comedone extractor or a small sterile needle. The authors rarely use cryotherapy for treatment unless the patient is an older child and lesions are few in number. Herpes simplex virus (HSV) infections are classified either as primary or recurrent. There are two types of HSV HSV-1 is usually associ-

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ated with oral and other nongenital infection, and HSV-2 is usually associated with genital infection. The most common presentation of primary herpes simplex infection in children is herpetic gingivostomatitis. Systemic symptoms may occur before the appearance of vesicles in and around the oral mucosa, which quickly become eroded and ulcerated. Primary herpetic infection may occur in the eye, with purulent conjunctivitis and vesicles and erosion of the cornea. Primary genital herpes presents as vesicles with erosions and ulceration on the external genital skin or genital mucosa. It may be accompanied by systemic symptoms. Herpetic whitlow is another presentation of primary herpes. In this case, the virus is inoculated into the skin of one or more fingers and can cause a painful vesicular eruption with swelling. Treatment for all forms of primary herpes infection is usually symptomatic, but oral antiviral medications may be prescribed to shorten the course of more severe disease and for immunocompromised patients. Recurrent HSV presents near the site of primary infection as grouped vesicles on an erythematous base. Lesions are usually asymptomatic but may be preceded by a burning or tingling sensation in the affected area. Again, treatment is symptomatic, but antiviral prophylaxis may be considered for patients with frequent recurrences. Patients must also be advised that the disease is infectious, and even in the absence of obvious lesions, viral shedding may occur. Many viral infections are accompanied by generalized erythematous rashes that are known as exanthems. The primary lesions may be macules, papules, vesicles, pustules, or petechiae. Some exanthemous eruptions have classic clinical features, but most appear nonspecific. Viral infections that are classically associated with exanthems include varicella, measles, rubella, erythema infectiosum, and roseola infantum. Other viruses that are commonly associated with exanthems include echoviruses, coxsackieviruses, reoviruses, and Epstein-Barr virus. Drug eruptions should be considered in the differential diagnosis of the more nonspecific viral exanthems. Treatment is symptomatic, with low-potency topical steroids, moisturizers, and antihistamines. Bacterial Infections of the Skin

Two common childhood bacterial infections of the skin are impetigo and folliculitis. Impetigo may be caused by staphylococci, streptococci, or both. The infection begins as small erythematous macules, commonly on the skin near the nose and mouth, which then progress to blisters. The blisters break easily to release a serous discharge, which then dries to form a honey-colored crust. In superficial infections, topical antibiotics such as mupirocin (Bactroban) may be adequate treatment. Systemic antibiotics, however, that appropriately cover the involved organisms may be required. Untreated impetigo may take several weeks to resolve, with spreading and development of new lesions in the meantime. The

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nose is a reservoir for staphylococci in asymptomatic individuals, and thus intranasal mupirocin may be used for prophylaxis in patients who have recurrent impetigo and in their household contacts. Folliculitis is an infection of the hair follicles. The most frequently isolated organism is Sfaphylococcus aureus. Clinically, folliculitis appears as follicular erythematous papules and pustules. For treatment, the patient may be advised to bathe with an antibacterial soap and apply topical antibiotics such as clindamycin (Cleocin T). In persistent or recurrent cases, systemic antibiotics may be required. A swab of one of the lesions should be sent for culture before initiation of systemic antibiotic therapy. Superficial Fungal Infections of the Skin There are three common forms of superficial fungal infection. Dermatophytosis, or tinea, is named according to the site of involvement. Tinea capitis, tinea corporis, tinea manum, tinea pedis, tinea cruris, tinea barbae, and tinea unguum (onychomycosis)may be seen in children and adolescents. Tinea versicolor and candidiasis are the other two forms of superficial fungal infections. Tinea capitis is rarely seen after puberty, whereas tinea pedis, onychomycosis, and tinea cruris are usually seen only in postpubertal patients. Clinical appearance varies with the location of the lesion, but classically there is an annular erythematous patch with a leading edge of scale. Tinea pedis may present only with scale on the lateral aspects of the feet or maceration between the toes. Infected nails are usually dystrophic, are yellowish, and have subungual debris. Onychomycosis is more often than not associated with tinea pedis. Clinical suspicion of tinea infection should be confirmed with a KOH preparation. If the KOH preparation is equivocal, fungal culture may be sent. Treatment with topical antifungal preparations is usually sufficient for all forms of tinea except for tinea capitis and onychomycosis. For these forms of tinea, systemic therapy must be administered for proper treatment. When making a decision regarding which topical antifungal to use for treatment of the other forms of tinea, the authors recommend avoiding the use of a combination antifungal/topical steroid such as clotrimazole l%/betamethasone dipropionate (Lotrisone). The disadvantage of such treatment is that topical steroids allow further growth of the organisms, and long-term use of such preparations may lead to the unforeseen side effects associated with topical steroids. Arthropod Infections of the Skin Scabies is caused by the mite Sarcoptes scabiei. The disease is a contagious infestation of the skin. Clinically, it is an extremely pruritic eruption of papules, vesicles, and, classically, burrows. The picture may

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be distorted by secondary changes of excoriation, crusting, eczematization, and superinfection. Older children and adults usually have lesions in the finger webs, flexural areas, beltline, and anogenital areas. In younger children, however, lesions are commonly found on the palms and soles and head and neck area. Infants can have a widespread eruption. Some children may develop nodular scabies, with persistent reddish brown nodules that represent a hypersensitivity reaction to the mite. Diagnosis may be made based on the combination of a history of persistent itching and clinical presentation and a history of itching in any close contacts of the patient. Diagnosis may be confirmed with a scabies preparation. The infestation is easily treated with topical preparations such as lindane (Kwell) and permethrin 5% cream (Elimite). The authors prefer permethrin because of reports of neurotoxicity of lindane, especially in children? 5, 7, 8, 12, 13, 15, l7 and conclusive evidence that the risk of toxic effects is lower for permethrin than for lindane.6 Careful instructions should be given to the patient or parents regarding the proper use of the preparation, and all household contacts should be treated. We also recommend washing in hot water all clothing that has been worn in the 2 days before treatment and all bed linens because mites can survive as long as 48 hours off of the human body. Care must also be taken to explain that even after treatment, pruritus may persist for several weeks. This persistent pruritus is due to a hypersensitivity reaction to the mite and should be treated symptomatically with antihistamines and mild topical corticosteroids. There are three types of lice, or pediculosis. Head and body lice infestations are caused by Pediculus humanus capitis and Pediculus humanus corporis. Head lice are commonly spread by sharing infested grooming items, and body lice are spread by close contact and wearing infested clothing. Pediculosis capitis is the most common form of lice. Pruritus of the scalp is the main complaint. On examination, nits (ova of lice) can be seen as tiny whitish oval bodies firmly attached to the hair shaft. If identification is in doubt, the hair may be cut and studied under the microscope for clarification. Pediculosis corporis is also associated with intense itching. The lice live in clothing or bedding, using the skin of the human host only for nutrition. Sometimes the nits can be seen firmly attached to the seams of clothing of affected patients. Phthirus pubis is the third type of lice. It causes pediculosis pubis, which also presents with pruritus of the involved area. The parasite and its nits are usually found on pubic hairs but may also inhabit other body hair. The authors prefer permethrin preparations over lindane for the treatment of lice, for the reasons discussed in the treatment of scabies. With regard to the treatment of head lice, the authors have observed that there is an increasing rate of resistance to over-the-counter preparations such as pyrethrin (RID) and permethrin 1% rinse (Nix). In these cases, the authors recommend application of permethrin 5% cream to the scalp once weekly for 2 weeks. If this treatment fails, a one-time liberal application of petroleum jelly to the scalp may be successful.

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PAPULOSQUAMOUS AND ECZEMATOUS DISORDERS Psoriasis is described as erythematous plaques with adherent silvery scale, distributed over the extensor surfaces of the extremities. Some variants of psoriasis should be kept in mind. Guttate psoriasis presents with the acute development of small droplike psoriatic plaques scattered over the trunk and proximal extremities. It is thought to be associated with streptococcal infection, and appropriate antibiotics sometimes help the acute flare. Inverse psoriasis, which affects mostly the flexural surfaces of the skin, is different from classic psoriasis not only in location, but also with regard to the absence of scale because the lesions are moist by virtue of their presence on flexural skin. Thus, if a diaper dermatitis does not respond to usual treatments, inverse psoriasis should be considered in the differential diagnosis. Atopic dermatitis is one of the most common skin disorders seen in children and constitutes one arm of the atopic triad. The other two components are allergic rhinitis and reactive airways disease. Atopic skin is generally dry and pruritic. With acute flares, scaly erythematous patches develop. In young infants, the face is the most common site of involvement. Closer to 1year of age, the extensor surfaces of the extremities tend to have greater involvement. In older children, teenagers, and adults, the flexural surfaces of the extremities, the face, and the neck are more involved. The basic principle behind long-term management of the skin of atopic patients is the frequent use of bland emollients. Creams and ointments are much more effective than lotions. Patient education should include instructions on daily bathing for a limited time in lukewarm water with a mild moisturizing soap or nonsoap cleanser. Emollients should be applied immediately after bathing while the skin is still moist, then several more times during the day. Another important facet of the management of atopic patients is education about environmental control. Antihistamines also have a role in the control of symptoms of atopy. The newer nonsedating antihistamines such as loratadine (Claritin) and cetirizine (Zyrtec) may help to provide daily relief if more traditional antihistamines are too sedating for use during the day. Topical corticosteroids should be reserved for the treatment of acute inflammation in atopic dermatitis. Severity, sites of involvement, and the age of the child should be considered in the selection of strength and vehicle of the steroid preparation. Evidence of bacterial superinfection should also be watched for, and appropriate antibiotics should be prescribed when necessary. The authors’ approach to the atopic patient is outlined in Table 2. Keratosis pilaris is often seen in patients with an atopic tendency. It first presents in early childhood and usually persists through the teenage years. Follicular hyperkeratosis may be present on the cheeks, outer aspects of the extremities, trunk, and buttocks. Treatment includes the frequent use of emollients and lactic acid preparations, such as lactic acid 12% (Lac-Hydrin). Pityriasis alba is a common cutaneous disorder of childhood. It

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Table 2. APPROACH TO ATOPIC DERMATITIS Chronic Management Short daily baths with lukewarm water and minimal use of moisturizing cleansers Frequent thick emollients (avoid lotions), especially after washing skin Antihistamines for pruritus Environmental control Acute Flares Low-potency to midpotency topical steroids 2 x / d Emollients over topical steroids and frequently as needed Antihistamines for pruritus Antibiotics for superinfection

presents as discrete hypopigmented, sometimes mildly scaly patches, most commonly on the face or arms. Reassurance and lubrication are the main components of treatment. A mild topical steroid may be used if the lesions are pruritic, and sun exposure usually results in repigmentation in several weeks. Seborrheic dermatitis presents as erythematous patches with greasy yellow scale on skin that has the highest concentration of sebaceous glands. Thus, seborrheic dermatitis is commonly seen in the scalp, near the eyebrows, in the nasolabial folds, and in the sternal region. The condition occurs usually in infants, adolescents, and postpubescent individuals because of increased sebaceous gland activity. In infants, involvement of the scalp is sometimes called cradle cap. Seborrheic dermatitis can also present in the diaper area in infants (Fig. 1). Rarely, diffuse involvement of the skin occurs. Low-potency topical steroids are usually effective in the treatment of seborrheic dermatitis. For scalp involvement, an antiseborrheic shampoo containing sulfur or salicylic acid is useful. For particularly thick scale that can occur especially in infancy, mineral oil may serve to help loosen the scale before shampooing. Several entities may be considered in the differential diagnosis of diaper dermatiti~.'~ Primary irritant diaper dermatitis results from constant contact of the skin with moist diapers. Examination reveals erythematous, sometimes macerated patches in the diaper area, distinctly sparing the skin folds. Treatment options include providing a barrier with diaper creams with every diaper change, frequent diaper changes, and low-potency topical steroids to help decrease inflammation. Because macerated skin is prone to developing superinfection with yeast, the authors suggest mixing nystatin (Mycostatin) cream with a low-potency topical steroid in the treatment of irritant contact dermatitis. The other common category of diaper dermatitis is primary yeast infection with Candida albicans. Presentation is similar to that seen in the irritant form, but skin folds are involved and satellite lesions may be observed. Satellite lesions refer to erythematous macules and papules that are seen scattered over the thighs and abdomen. Treatment is with nystatin cream.

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Figure 1. Seborrheic dermatitis with significant hypopigmentation in the diaper area of an African-American infant.

Nickel dermatitis is one of the most common causes of allergic contact dermatitis. Nickel may be found in earrings and snaps or metal buttons, especially on jeans. Thus, a common presentation of nickel dermatitis is with scaly erythematous patches on the earlobes or around the umbilicus. Hypersensitivity to nickel lasts for years, and thus the main component of treatment is avoidance of nickel-containing substances. These may be coated with clear nail polish or covered with cellophane tape if they must be used. Topical steroids may be used to treat the dermatitis. Pityriasis rosea presents acutely with pruritic brownish to erythematous oval patches with a collarette of scale, classically in a Christmas tree distribution on the trunk. The herald patch precedes this eruption by approximately 1 week. This patch is clinically similar to the patches in the acute eruption but is usually larger. It most commonly occurs on the trunk. Pityriasis rosea is thought to have a viral cause, although no virus has yet been isolated. It usually resolves in 6 to 8 weeks, and treatment is symptomatic, with antihistamines and topical antipruritics. Reassurance as to the benign, self-limited nature of this condition is an important part of management. Lichen striatus is another benign, self-limited eruption. It presents unilaterally with tiny discrete and confluent lichenoid papules, usually

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on an extremity, in a linear pattern (Fig. 2). The color of the papules varies from patient to patient, but it is usually pink or sometimes hypopigmented in darker-skinned individuals. Lichen striatus is seen in early childhood and resolves without treatment in 1 to 2 years. Reassurance is the treatment of choice. INFLAMMATORY SKIN CONDITIONS

Acne is the presenting complaint of many adolescent and young adult patients. The presentation of acne can vary from mild comedonal acne to severe nodulocystic acne. There are basically two types of acne: noninflammatory and inflammatory. The open (blackhead) or closed (whitehead) comedone is the basic lesion of noninflammatory acne, and inflammatory lesions such as papules, nodules, and cysts all arise from comedones. Comedonal acne can usually be successfully treated with topical keratolytics such as retinoids. If there is a mild inflammatory component, benzoyl peroxide preparations may be added. If the inflammatory component is more advanced, topical antibiotics, or with even more inflammation, oral antibiotics, may be added. It is imperative that the physician prescribe a treatment regimen that the patient will follow. The physician must also explain to the patient that treatment should be continued for at least 6 weeks before visible results can be expected. This point is important to make so that patients do not have unrealistic expectations and abandon treatment regimens before the time period in which results can reasonably be expected. For severe nodulo-

Figure 2. Lichen striatus in a young child.

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cystic acne or other inflammatory acne .unresponsive to adequate trials of the above-mentioned medications, oral isotretinoin is an option. This medication has many side effects, the most devastating of which is teratogenicity if taken during pregnancy, and thus it should be prescribed with caution only in appropriately selected patients. Furthermore, prescription guidelines and appropriate monitoring should be followed. Table 3 outlines the author’s general approach to the patient with acne. Another important aspect of acne treatment is understanding that although acne is for the most part a cosmetic problem, what may be perceived as a minor degree of severity by the physician may be significant to the patient. The major morbidity of acne arises from the psychological impact that it can have on a patient’s self-esteem and psychological well-being, especially in the vulnerable period of adolescence. Urticaria, commonly known as hives, occurs in many patients for a variety of reasons. The lesions present acutely and are transient in nature, with individual lesions resolving within 24 hours. They appear as well-demarcated erythematous wheals that are intensely pruritic. If an inciting agent can be identified, attempts should be made to eliminate exposure. Possible causes are listed in Table 4. Symptomatic treatment is with antihistamines. In general, extensive laboratory evaluation of acute urticaria is not indicated in the otherwise healthy patient. Granuloma annulare is a relatively common disorder that may be seen at any age, although children and young adults are more commonly affected. Classically, grouped erythematous to skin-colored papules and

Table 3.APPROACH TO ACNE Comedonal (noninflammatory) Topical retinoids applied qhs Mild inflammatory Topical retinoids applied qhs Topical benzoyl peroxide formulated as facial washes and gels Topical antibiotics Clindamycin formulated as solutions and gels for oily skin and lotions for dry skin Erythromycin Moderate inflammatory Topical retinoids applied qhs Systemic antibiotics Tetracycline Doxycycline Minocycline Observe for hyperpigmentation with long-term use Erythromycin Trimethoprim/sulfamethoxazole (Bactrim) Severe inflammatory (nodulocystic) Systemic isotretinoin 80% of patients have an excellent response with a 20-wk course Avoidance of pregnancy during use is imperative qhs = Every hour of sleep.

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Table 4. CAUSES OF URTICARIA* Allergic Food Drugs Insect bites Contact

Physical Pressure Cholinergic Aquagenic Solar Cold

‘Idiopathic in S O % of cases.

nodules slowly involute centrally, with peripheral extension, to form rings. Subcutaneous forms can-be seen on the extremities. Granuloma annulare usually resolves spontaneously in several months to several years, and if the lesions do not bother the patient, no specific treatment except for reassurance is necessary. If the patient desires treatment, intralesional triamcinolone injections are sometimes useful. This disorder can be easily distinguished from dermatophyte infections because of the absence of scaling in granuloma annulare (Fig. 3). Alopecia areata is a disease of the hair that usually presents first in childhood or young adulthood. Most commonly, patients complain of one or several well-circumscribed patches of hair loss, usually in the scalp but also sometimes in the beard area of adolescent boys and young men. On closer examination, the patches of hair loss are not scarred, and follicles are still visible. Exclamation point hairs may be seen around the margin of alopecic patches. Nail pits may also be observed. Loss of all scalp hair is termed alopecia totalis, and loss of all body hair is termed alopecia universalis. Autoimmune processes such as thyroid disease are sometimes associated with alopecia areata. If indicated by the history and physical examination, screening laboratory work should be done. The course and prognosis of alopecia areata are extremely variable but sometimes may be altered with treatment. Treatment of one or a few localized patches may be attempted with intralesional injections of triamcinolone. In patients that cannot tolerate intralesional injections, highpotency topical steroids may be useful. In selected cases that are unresponsive to local steroid treatment, systemic steroids may be effective. The potential risk-to-benefit ratio of systemic steroid treatment must be carefully weighed. The major differential diagnosis of alopecia areata to consider when examining a patient with patchy hair loss is trichotillomania (Fig. 4). It is most commonly seen in school-aged children and adolescents. Patients consciously or subconsciously pull their hair out. In contrast to alopecia areata, the patches of hair loss are not well circumscribed, and within the patches there are broken hairs and hairs of varying lengths. The management of trichotillomania may be difficult because patients sometimes do not realize or do not admit that it is self-induced. A good rapport with the patient is helpful, but sometimes professional psychological intervention may be necessary.

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Figure 3. A, Tinea faciale, with pronounced epiderma change. B, Granuloma annulare, with a more dermal component.

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Figure 4. A, Well-circumscribed patches of hair loss in alopecia areata. B, Patches of alopecia with angular borders and hairs of varying lengths in trichotillomania.

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Vitiligo is a pigmentary disorder that presents with well-defined depigmented macules and patches of varying shapes and sizes. Classically, islands of repigmentation are evident within the depigmented patches. Onset is usually in young adulthood, but it may occur in children as well. There may be association with other autoimmune diseases, such as thyroid disease and diabetes mellitus. If the patient’s history and physical examination suggest other autoimmune problems, laboratory work should be done to screen for these entities. The course of vitiligo is variable. There is the possibility of repigmentation of patches that occur in hair-bearing areas, but sites lacking hair follicles tend not to repigment with or without treatment; repigmentation occurs primarily as a result of migration of melanocytes from the hair bulb.’, In patients with limited involvement, potent topical steroids may be tried for 3 to 4 weeks; if no results are apparent after this time period, continued use is not likely to result in any response. For widespread disease, a regimen of oral psoralens followed by controlled ultraviolet A light exposure may aid in repigmentation. Only motivated patients should undertake this treatment because it requires a long period of time and is not always successful. The cosmetic aspect of the disease is often the most devastating to the patient, and in addition to therapeutic options, the physician must provide emotional support. Several good cosmetic camouflages are available as well. The authors recommend Dermablend, which is available in most department stores. Children may present to the physician’s office with cutaneous evidence that they are victims of child abuse or neglect. Clustering of bruises, adult human bite marks, and bruises and abrasions on the face and perineal areas should alert the physician. Burns, especially in the shape of the end of a cigarette, are also common evidence of child abuse. An unusual number of scars may be noted. Lesions can be sharply defined and in the shape of the instrument used to inflict injury, and often bruises are in a fingertip pattern. Multiple other injuries, such as fractures, should heighten the physician’s suspicion. A child’s lack of affection toward the caretaker and ready willingness to be away from the caretaker during examination are also clues to child abuse or neglect. If strong evidence of child abuse or neglect is present, appropriate social agencies should be contacted. If the physician suspects that the child may be in immediate danger, hospitalization of the child is imperative for protection and further assessment. NEOPLASTIC DISEASES OF THE SKIN

Melanocytic nevi, or moles, are common in children and adults. There are three types of benign melanocytic nevi. Flat brown moles are usually junctional nevi, and histologically the nests of nevus cells are present at the dermal-epidermal junction. Intradermal nevi are usually nodular and sometimes papillomatous; their color can vary from skincolored to tan to dark brown. Histologically, intradermal nevi have nests

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of nevus cells within the dermis only. Compound nevi histologically demonstrate nests of nevus cells both at the dermal-epidermal junction and within the dermis; clinically, they may be difficult to distinguish from intradermal nevi, although they are usually less elevated. When examining moles, it is important to keep in mind the ABCDs of melanoma (see Table 5). With a combination of these features, skin biopsy should be done to rule out malignancy. Congenital melanocytic nevi are present at birth, and they are generally classified as small, intermediate, and giant or large. Small congenital nevi are less than 1.5 cm in diameter, giant congenital nevi are greater than 20 cm2 in area, and intermediate ones fall in between.'l Giant congenital nevi are often unevenly pigmented with shades of brown-toblack, may have a hairy component, and are often cosmetically devastating. Controversy exists as to the management of these lesions. A major issue is their malignant potential. The true lifetime risk for the development of melanoma in giant congenital nevi is approximately 10%. Our preference is to observe the child on a periodic basis, with more frequent evaluation in the first few years of life because early childhood is the period during which there is the greatest potential for malignant change.lO, l6 Biopsies should be performed in suspicious areas. Total excision with tissue expanders and skin grafting is another option. We also favor observation of small and intermediate-sized congenital melanocytic nevi, unless they are in locations that are difficult to observe, such as the scalp or perineal areas, until the child is mature enough to tolerate excision with local anesthesia. It is usually after adolescence that these lesions undergo malignant transformation; with a risk of 0.8% to 4.9% for malignant transformation of small congenital nevi in patients who live to the age of 60 years.I8 In the management of congenital melanocytic nevi, it is important to establish a good rapport with the family and with the patient when he or she is mature enough. Parents and mature children should participate in management decisions. Hemangiomas are the most common tumor in children, occurring in about 10% of children. They are vascular proliferations that occur more commonly in girls than in boys and more commonly in the head and neck area than over the rest of the body. The older terminology of capillay hemangiomas, strawber y hemangiomas, and cavernous hemangiomas should no longer be used. Descriptions should now be limited to superficial versus deep. Hemangiomas usually become evident within the first few weeks of life and undergo rapid proliferation in the first few months. Most reach maximal growth by 1 year of age then start to involute slowly. A decrease in redness and eventual graying of the lesion occur during involution (Fig. 5). When discussing prognosis with parents, they can be told that 50% of lesions resolve by 5 years of age, 70% of lesions resolve by 7 years of age, and 90% of lesions resolve by 9 years of age. Superficial lesions may resolve completely, whereas those with a deeper component may resolve partially with the need for later corrective cosmetic surgery. Rapidly proliferating hemangiomas that are of concern

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Figure 5. Involuting hemangioma.

should be treated with prednisone at a dose of 2 to 3 mg/kgld over a 3to 4-week period, with a slow taper. Immunizations should be held until the patient has been off prednisone for at least 1 month. For rapidly proliferating hemangiomas that do not respond to systemic steroids or are compromising vital structures, treatment with interferon alfa-2a has been successful in some patients. The pulsed dye laser can be used in the treatment of the superficial component of hemangiomas, and its use is especially successful in the treatment of the ulcerations of rapidly proliferating lesions. Solitary mastocytomas are the most common form of mastocytosis. They appear as one or a few tan plaques or nodules that may have a slightly erythematous hue. They present at birth or in early infancy. An aid to diagnosis is that these lesions tend to urticate when stroked firmly; this finding is called Davier’s sign (Fig. 6). Biopsy is confirmatory and shows collections of mast cells. The lesions are generally asymptomatic, and parents can be reassured that spontaneous resolution occurs within several years. Keloids are most easily described as scars that have proliferated beyond the boundaries of the original injury. They present as firm fibrotic nodules or plaques at sites of previous trauma that the patient may or may not recall. Dark-skinned individuals are more prone to

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Figure 6. Solitary rnastocytoma with positive Darier’s sign.

developing keloids than light-skinned individuals. Common locations are the earlobes in people who have pierced their ears and the trunk. Lesions are usually asymptomatic, although they may be slightly pruritic in the proliferative phase, and cosmetic issues are what prompt most patients to seek treatment. For smaller lesions, up to a few centimeters in diameter, intralesional triamcinolone may serve to soften and flatten the tissue. Larger lesions may be excised in combination with intralesional triamcinolone injections. CONCLUSION

Many patients with dermatologic conditions first present to their general practice physician. Summarized in this article are skin diseases that physicians who treat children would be aware of. A general approach to management of these conditions has also been outlined. If the initial management of common dermatologic conditions is not successful, differential diagnoses must be explored further, and referrals to specialists should be made when appropriate. There are some less commonly encountered entities that may present to the general practice physician with dermatologic manifestations. These are outlined in Table 5.

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Table 5. DERMATOLOGIC MANIFESTATIONS OF LESS COMMON ENTITIES THAT MAY PRESENT IN THE OFFICE SETTING NeurocutaneousSyndromes Neurofibromatosis Cafe au lait macules Axillary freckling Neurofibromas Tuberous sclerosis Hypopigmented macules (ash-leaf spots) Angiofibromas (adenoma sebaceum) Shagreen patches (connective tissue nevi) Periungual fibroma (Koenen’s tumors) Heritable Disorders lchthyosis Genodermatosis characterized by dry scaly skin Four classes lchthyosis vulgaris (most common) X-linked ichthyosis Epidermolytic hyperkeratosis Lamellar ichthyosis Epidermolysis bullosa Several clinical variants of this group of inherited disorders Onset at birth or in early childhood All have in common blistering that is spontaneous or results from friction or trauma Erythropoietic protoporphyria Burning and itching of sun-exposed skin Development of erythematous plaques and vesiculation on sun-exposed skin Chronic changes with dyspigmentation and thickening of skin in affected areas lncontinentia pigmenti Four cutaneous stages Vesiculobullous Verrucous Hyperpigmented Hypopigmented X-linked dominant inheritance (thus lethal in males) Basal cell nevus syndrome Multiple basal cell carcinomas Milia and comedonal lesions on the face Benign cutaneous tumors Epidermal cysts Lipomas Fibromas Cafe au lait macules Multiple nevi Ectodermal dysplasia Inherited group of defects involving skin and appendages Three categories Hidrotic ectodermal dysplasia Anhidrotic ectodermal dysplasia Aplasia cutis Other Entities Pityriasis lichenoides Recurrent crops of macules, papules, and vesicles Develop necrosis and crusting Chronic forms characterized by distinctive hypopigmented macules Henoch-Schonlein purpura Palpable purpura in symmetric distribution over buttocks and extremities Lesions occur in crops Table continued on following page

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Table 5. DERMATOLOGIC MANIFESTATIONS OF LESS COMMON ENTITIES THAT MAY PRESENT IN THE OFFICE SETTING (Continued) Histiocytosis Scaly erythematous patches (similar to seborrheic dermatitis) Petechiae and purpura Vesicles and reddish brown papules Should be considered when common diagnoses do not respond to standard therapy Melanoma Suspicious mole that has been changing, bleeds, or is ulcerated ABCDs of melanoma Asymmetry Border (scalloped, irregular) Color (various shades of black, brown, red, or blue) Diameter (>6 mm) Acquired immunodeficiency syndrome Severe and unusual skin infections Cutaneous neoplasms Severe seborrheic dermatitis

Physicians who manage skin disease in children should always keep in mind the age and maturity level of the child when making decisions regarding evaluation and treatment, especially with regard to skin lesions that are benign and self-limited. Every attempt should be made to treat the child’s skin condition appropriately without unnecessarily traumatizing the patient. References 1. Arrunategui A, Arroyo C, Garcia L, et al: Melanocyte reservoir in vitiligo. Int J Dermatol 33:484487, 1994 2. Bauman C, Francis JS, Vanderhooft S, et al: Cimetidine therapy for multiple viral warts in children. J Am Acad Dermatol 35:271-272, 1996 3. Boffa MJ, Brough PA, Ead R D Lindane neurotoxicity [letter]. Br J Dermatol 133:1013, 1995 4. Cui J, Shen LY, Wang GC: Role of hair follicles in the repigmentation of vitiligo. J Invest Dermatol97410-416, 1991 5. Davies JE, Dedhia HV, Morgade C, et al: Lindane poisonings. Arch Dermatol 119:142144, 1983 6. Franz TJ, Lehman PA, Franz SF, et al: Comparative percutaneous absorption of lindane and permethrin. Arch Dermatol 132:901-905, 1996 7. Friedman SJ: Lindane neurotoxic reaction in nonbullous congenital ichthyosiform erythroderma. Arch Dermatol 123:10561058, 1987 8. Gamma benzene hexachloride (Kwell and other products) alert. FDA Drug Bull 6:28, 1976 9. Illig L, Weidner F, Hundeiker M, et al: Congenital nevi less than or equal to 10 cm as precursors to melanoma: 52 cases, a review, and a new conception. Arch Dermatol 121:1274-1281, 1985 10. Kaplan EN: The risk of malignancy in large congenital nevi. Plast Reconstr Surg 53421428, 1974 11. Kopf AW, Bart RS, Hennessey P: Congenital nevocytic nevi and malignant melanomas. J Am Acad Dermatol 1:123-129, 1979

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12. Lee 8,Groth P: Scabies: Transcutaneous poisoning during treatment [letter]. Pediatrics 59:643, 1977 13. Matsuoka LT Convulsions following application of gamma benzene hexachloride [letter]. J Am Acad Dermatol 5:98-99, 1981 14. Orlow SJ, Paller A: Cimetidine therapy for multiple viral warts in children. J Am Acad Dermatol 28:794-796, 1993 15. Pramanik AK, Hansen RC: Transcutaneous gamma benzene hexachloride absorption and toxicity in infants and children. Arch Dermatol 1151224-1225, 1979 16. Quaba AA, Wallace AF: The incidence of malignant melanoma (0-15 years of age) arising in ”large” congenital nevocellular nevi. Plast Reconstr Surg 78174-179, 1986 17. Ramchander V, Cameron ES, Reid H F Lindane toxicity in an infant. West Ind Med J 40:41-43, 1991 18. Rhodes AR, Melski J W Small congenital nevocellular nevi and the risk of cutaneous melanoma. J Pediatr 100:219-224, 1982 19. Singalavanija S, Frieden IJ: Diaper dermatitis. Pediatr Rev 16:142-147, 1995 20. Yilmaz E, Alpsoy E, Basaran E: Cimetidine therapy for warts: A placebo-controlled, double-blind study. J Am Acad Dermatol 34:1005-1007, 1996

Address reprint requests to James F. Nigro, MD Dermatology Service Texas Children’s Hospital 6621 Fannin, MC 3-3315 Houston, TX 77030