ADULT SKIN DISEASE IN THE PEDIATRIC PATIENT

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PEDIATRIC DERMATOLOGY

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ADULT SKIN DISEASE IN THE PEDIATRIC PATIENT Renee Howard, MD, and Arline Tsuchiya, MD

It is well known that cutaneous diseases such as atopic dermatitis occur in childhood, and dermatologists have no difficulty recognizing and managing these disorders in children; however, the incidence of many skin diseases peaks in adulthood. These diseases may be difficult to recognize when they present in childhood, as their clinical features and natural history may be distinct in children. In addition, they may be managed differently in the pediatric patient. For example, methotrexate is used more cautiously in children than in adults with psoriasis. The differential diagnosis of inflammatory skin disease in children is unlike that in adults, and it may be more problematic to do diagnostic skin biopsies in a young child. Therefore, it is important that dermatologists recognize ”adult” skin disease in the pediatric patient, and know how to appropriately treat young patients with these disorders. This article will review the epidemiology, clinical features, differential diagnosis, and treatment of the following ”adult” skin diseases in children: psoriasis, lichen planus, Sweet’s syndrome, rosacea, and mycosis fungoides. Finally, distinctive features of lichen sclerosus and immunobullous diseases in childhood will be briefly discussed.

is characterized by erythematous papules or plaques with silvery white scale. The incidence is 1% to 3% of the general population.28,87 Psoriasis is less common in blacks, Japanese, and Native Americans than in whites.’,29, Io4 Psoriasis in the pediatric age group is not rare. In large scale reviews of psoriasis patients, almost 40% developed psoriasis prior to the age of 20 years, and 10% prior to the age of 10 years.22,23, 29 Psoriasis in infancy has been well documented.25,89 A family history of psoriasis can be obtained from about 35% of patients.29The specific mode of inheritance is unknown, but psoriasis is most likely a polygenetically inherited disease. Distinctive HLA types have been seen in patients with psoriasis: HLA types Cw6 and DR7 are linked to early-onset psoriasis and HLA-Cw2 to late-onset psoriaS ~ SRegardless, . ~ ~ HLA typing cannot be used to diagnose psoriasis, or for genetic counseling of families with psoriasis. Recent localization of genes involved in psoriasis at the distal end of the long arm of chromosome 17 may contribute to identification of genes involved in psoriasis s~sceptibility.~~

PSORIASIS

Skin lesions of psoriasis in children are identical to those in adults, and all clinical subtypes can be seen. Plaque type psoriasis is

Psoriasis is a well-described chronic skin disorder seen in both adults and children and

Clinical Features

From the Division of Pediatric Dermatology, Children’s Hospital Oakland, Oakland (RH); Department of Dermatology, University of California at San Francisco, San Francisco (RH); and the Southern California Permanente Medical Group (AT), Garden Grove, California

DERMATOLOGIC CLINICS VOLUME 16 * NUMBER 3 *JULY 1998

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Figure 1. Symmetrical, discrete, red plaques with silvery scale on the anterior legs of a 12-year-old boy with plaque-type psoriasis.

the most common form of psoriasis seen in children, and is characterized by well-demarcated, thick, silvery scaly red plaques in a symmetrical distribution on extensor surfaces In many studies, the scalp is the (Fig. 1).2,7,87 most frequently involved area; it is the most common site of onset in 40% to 60% of patients prior to 20 years of age.28,47, 87 Scalp psoriasis may be mistaken for seborrheic and atopic dermatitis, or tinea capitis. Psoriasis in the diaper area can be the initial presentation of psoriasis in infancy (Fig. 2).24,26 Genital

involvement may persist into childhood and adolescence (males > females). In girls, the mons pubis, labia majora, and inguinal folds are affected, while in boys, the penis is usually involved.26Psoriasis in the diaper area and groin is often confused with candidal infection and irritant contact dermatitis. Nail involvement is characteristic of adult psoriasis, but is seen just as frequently in children.27Nail changes include pits, yellow discoloration ("oil spots"), onycholysis, and subungual debris. In children under the age

Figure 2. Confluent erythema in the diaper area of a infant presenting with psoriasis at 10 months of age. Scaly red papules were also present on the extensor surfaces of her arms and legs, but she was referred for diaper rash that was unresponsive to the usual treatment.

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of 18 years, nail involvement is seen in 7% to 40%; pitting is the most common abnormal-

it^.^^, 87, 90 The differential diagnosis includes lichen planus of the nail (which is very rare in children), onychomycosis, and posttraumatic nail changes. Guttate (drop-like) psoriasis is a common presentation in children in which small, round 1 to 3 mm scaly papules occur on the trunk and proximal extremities. This may occur 1 to 2 weeks after streptococcal pharyngitis or viral illness, and has also been described in association with perianal streptococcal d e r m a t i t i ~ . 133 ~ ~ ,The differential diagnosis of guttate psoriasis includes nummular dermatitis and pityriasis rosea. Pustular psoriasis is an uncommon subtype in both adults and children, and may be localized or generalized (von Zumbusch). The generalized form has been reported in children, and is characterized by widespread eruption of sterile pustules and systemic symptoms of fever, malaise, and anorexia (Fig. 3).52,56, 139 An annular configuration may 60, 139 Children with genbe seen in ~hildren.~, eralized pustular psoriasis have a more benign course than adults.60,139 Pustular psoriasis may be misdiagnosed as a bacterial folliculitis, impetigo, or candidal infection. Psoriatic erythroderma is very rare in children and uncommon in Erythema

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over 90% of total body surface area is the prominent feature with less scaling than is seen in plaque-type psoriasis.12 Psoriatic arthritis is an inflammatory seronegative arthritis accompanied by psoriasis. Psoriatic arthritis in the first two decades of life is uncommon: the prevalence increases from the third to sixth decade, where it peaks.l13 A juvenile subset has been described with a peak of onset of 10 years that occurs more frequently in girls.46,lo9 Treatment

The majority of children have mild disease and can be treated with topical therapy; it is rare that one has to resort to systemic therapy when treating childhood psoriasis. Most of the topical agents and many of the systemic agents used in adult psoriasis are also used in childhood psoriasis. Options for treatment of childhood psoriasis are outlined in Table 1. Identifying and eliminating triggers of psoriasis in an individual child may sometimes be helpful. Triggering factors include infection (e.g., streptococcal), medications (e.g., systemic steroids, lithium, antimalarials, betablockers), and trauma.', 96, 133 The Koebner or isomorphic phenomenon (psoriasis developing in the area of trauma) is seen in psoria-

Figure 3. Pustular psoriasis on the lateral trunk of a 5-year-old boy who presented with fever and widespread tender, discrete, annular red plaques studded with superficial pustules. This child was referred to dermatology by the infectious disease service after multiple negative bacterial cultures and empiric antibiotics.

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Table 1. TREATMENT OF PSORIASIS IN CHILDREN Pediatric Use

Side EffectsIPrecautions

Topical Preparations Emollient Useful in mild disease, and good adjunct in more severe diseasea First line therapy for all subtypes of psoriasisll. 40 Topical steroid

Coal tar Anthralin Calcipotriol Tazarotene gel

Phototherapy UVB PUVA

Systemic Agents Methotrexate Etretinate Cyclosporine

Thick plaques Short contact therapy useful in children with large, thick plaques14o Used in children for limited plaques17,91 Limited, mild to moderate plaqueslZ8,lZ9 Not studied in children Useful in children with widespread pIaqueslz4 Limited use in childhood31.74, lZ2

None Tachyphylaxis Local side effects: atrophy, striae Systemic side effects: cataracts, growth impairment, adrenal suppression Irritation, staining, foul smell Irritation, staining Irritation Facial rash17 Irritation

Cost, inconvenience Concern over long-term risks of carcinogenesis, short term cataracts with systemic psoralens Cost, inconvenience

Useful for severe, erythrodermic, pustular Bone marrow and hepatotoxicity psoriasis and psoriatic arthropathy in childhoods0,67. 13’ lo6, 117, lZ7 Useful for pustular psoriasis in Pruritus, cheilitis, skin fragility, hair loss, musculoskeletal pain Bone toxicity if used long-term42.loo. lo8 Not used in children for psoriasis

sis as well as other skin diseases such as lichen planus (Fig. 4). Psoriasis can develop in areas of friction, scarring, or sunburn.12 The appearance of psoriasis in infancy in the diaper area is thought to be a manifestation of the Koebner reaction.24,26 Stress has been implicated as a triggering factor, but may be overrated in adults as well as ~ h i l d r e nOther .~ evidence suggests that stress is an important trigger in some patients, and for this population, stress reduction techniques such as hypnosis and biofeedback can be helpful.30 Topical Medications

Emollients

Emollients are a very important part of the skin care regimen of pediatric patients with psoriasis. Emolliation alone can improve mild cases of psoriasis.40In infancy and early childhood, bland, inexpensive emollients such as simple petrolatum work well. As the pre-adolescent and adolescent years approach, emollients (with or without keratolytics) with more cosmetic appeal can be used.

Corticosteroids Medium to high potency topical corticosteroids are effective in most cases of adult and childhood psoriasis11,*O; however, long-term use can lead to side effects such as atrophy, striae, and tachyphylaxis. If used over extensive areas, topical steroids may cause suppression of the pituitary-adrenal axis, growth delay, cataracts, and other systemic side effects. Use of the least potent topical corticosteroid to maintain control should always be emphasized, as well as tapering the strength of corticosteroids once control is achieved. For facial psoriasis, 1%hydrocortisone or tridesilone cream or ointment can be used. For plaques on the trunk and extremities, medium potency (triamcinolone 0.1% ointment or cream) to high potency (fluocinonide)topical corticosteroids are usually required. Scalp psoriasis can be treated with liquid forms of corticosteroids such as fluocinonide solution overnight, followed by shampoo with various preparations containing cortisone, tar, zinc, or salicylic acid. Other nonsteroid containing olive or mineral oil can be used to soften and loosen the scale in the scalp.

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dren.17,91 Adverse events include local irritation and minor facial rash.17 Tazarotene

Tazarotene is a topical retinoid gel that is useful in treatment of limited (< 20% BSA), mild to moderate psoriasis.12y Compared with fluocinonide 0.05% cream BID, tazarotene gel applied once daily was comparable in reducing plaque elevation, and its therapeutic effect was more prolonged.128Adverse effects are usually limited to local irritation. There is systemic absorption of the drug in low levels in approximately 50% of patients.lz9Tazarotene is not recommended for use in pregnant women or in women considering pregnancy. Its use in children has not been studied.

Figure 4. Lichen planus on the leg of a 4-year-old girl. Note linear array of papules on medial aspect, most likely due to Koebner (isomorphic) effect.

Coal Tar

These preparations have some value when used topically, and can be used in a cream base in combination with topical steroids, or in a liquid used in the bath. However, for children (and many adults) the odor, color, and staining properties make it a difficult treatment to accept, leading to problems with patient compliance. Anthralin

Ultraviolet Light

Most patients show improvement of psoriasis with sun exposure. Although natural sunlight is easier and less aggressive than artificial UV therapy, UVB phototherapy has been shown to be well tolerated and useful in treating psoriasis in children as young as 14 months.50,lz4 PUVA (oral psoralen and UVA) has been used for over 20 years for severe psoriasis; however, its use in children is controversial because of the increased long-term risk of squamous cell carcinoma.", 74, lz2 Systemic Agents

Anthralin is also a useful adjunct preparation, especially for treating larger, thick plaques. Short contact anthralin (applied for 30 minutes a day) has been used in children, with improvement in approximately 80%.140 However, unpleasant side effects such as staining and irritation occur in 20% of patients.

It is the exceptional child with psoriasis who requires systemic therapy. Methotrexate and the oral retinoids (etretinate) have been used in children with severe erythrodermic psoriasis, pustular psoriasis, or psoriatic arthritis.67,106, 117

Calcipotrio1

Although methotrexate has been used for over 35 years in the treatment of adult psoriasis, its use in childhood psoriasis is limited to a few reports.60,67, 137 Methotrexate was used successfully in a recent report of 7 children 3.5 to 16 years of age with severe psoriasis (3 with erythrodermic psoriasis, 2 with generalized pustular psoriasis, and 2 with recalcitrant psoriasis or psoriatic a r t h r ~ p a t h y ) . ~ ~

This vitamin D analog is supplied in cream, ointment, and liquid forms (for scalp application) and is best used for treatment of limited plaque type psoriasis (< 30% of body surface area [BSA]). It is comparable to betamethasone 17-valerate cream,I7 and its safety and efficacy have been demonstrated in chil-

Methotrexate

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Methotrexate was given in dosages of 0.2 to 0.4 mg/kg once a week. Control was achieved in 6 to 10 weeks, then the dosage was tapered by 2.5 mg of methotrexate per week until complete withdrawal. The total duration of therapy was 31 to 46 weeks. No laboratory abnormalities were detected and there was no effect on growth or secondary sex characteristics. No posttreatment liver biopsy was done. Three of the treated children had nausea and vomiting controlled with metoclopramide. Gastrointestinal side effects of methotrexate can be avoided if folic acid is given orally, 5 mg twice ~ e e k 1 y . I ~ ~

visible disorder on a child or adolescent’s psychosocial development is significant. Therefore, emphasis on education and emotional support for parents and patients is important so that they understand the rationale behind, and limitations of, treatment. Management should be conservative, but effective. Control of psoriasis can be achieved and recurrences can be treated with the proper care and support. LICHEN PLANUS

Lichen planus is an uncommon disease of skin and mucous membranes in both adults and children. The incidence peaks between Systemic Retinoids the ages of 30 and 60 years, and children Systemic retinoids (etretinate and isotreticomprise only 2% to 3% of reported cases.8 noin) in childhood have primarily been used Lichen planus in infancy is extremely rare, for the i~hthyoses.’~~ There are only a few but has been reported.58,lo2 reports of their use in childhood psoriasis.55* The etiology of lichen planus is unknown, lo6, 117, lZ7 The majority of these children had and genetic predisposition probably does not generalized pustular psoriasis and were play a major role. Familial lichen planus is treated with 0.25 to 1.5 mg/kg/day of etretiuncommon, with an incidence ranging from nate. Clearing in a series of five children with 65, 84 It involves younger about 1%to 11%.59T pustular psoriasis occurred within 3 weeks to persons and clinically appears as a more ex4 months after initiation of etretinate therapy. tensive disease with longer duration.65,73 CerLaboratory values in general remained nortain HLA types have been associated with mal. The most frequent side effects were prufamilial lichen planus (HLA DR, HLA B7, ritus, cheilitis, and skin fragility. Hair loss and HLA A3, HLA A28). However, HLA typing musculoskeletal pain were noted in 20% of has not proven helpful in predicting the inpatients 1 to 2 months after initiation of treatheritance of lichen planus thus far.73,131 ment; however, these side effects were dosage-dependent and reversible off etretinate.lo6 CIinical Features The risk of skeletal toxicity (premature epiphyseal closure, growth retardation, osteopoThe primary lesions of lichen planus are rosis, cortical hyperostosis) is of major consmall, violaceous, flat-topped polygonal papcern, and has been noted in children treated with retinoids over long periods of time.42,100,108ules that are usually intensely pruritic (see Fig. 4). The eruption may be generalized or However, short-term use of the retinoids to confined to a few areas, and may result in treat pustular flares of psoriasis in children marked postinflammatory hyperpigmentahas been reported to lack the risks of skeletal tion (Fig. 5). Childhood lichen planus may changes seen with long-term use.39 present in a more atypical fashion than adult lichen planus, including linear, annular, bulCyclosporine lous, and follicular variants.84The morpholCyclosporine has been shown to be effecogy and differential diagnosis of these varitive for treatment of psoriasis in adults. When ants are summarized in Table 2. the medication is tapered, relapses can ocLinear lichen planus is an uncommon clinicuts5 Its use in children with psoriasis has cal variant in adults, but has been described not been well studied. 58, 84 Hypertrophic lichen planus in children.57, has been reported in children as well.57Lichen planus atrophicus may be the result of resolvPrognosis ing hypertrophic lichen planus, with atrophy The majority of children with psoriasis do and only a few lesions. very well; however, the impact of a chronic, Actinic lichen planus is a variant usually

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Figure 5. Widespread marked postinflarnrnatory hyperpigmentation after generalized lichen planus on the back of same patient shown in Figure 4.

seen in the third decade of life, most commonly in residents of the Asian continent. Onset is in the spring and summer, with involvement of exposed skin. In a series of 16 patients with actinic lichen planus, 2 were children aged 13 and 16 years old.”’

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In lichen planopilaris (follicular lichen planus), lesions occur in a follicular pattern, usually in women and often associated with alopecia of the scalp. Clinically, it can resemble pseudopelade. This has also been described in children.18,84 Nail abnormalities occur in up to 10% of patients with lichen planus, but proven childhood lichen planus of the nails is rare. Nail involvement (nail dystrophy, atrophy, ridging, and pterygium formation) can occur without other skin manifestations of lichen 54 Some cases of idioplanus in ~hi1dren.I~. pathic atrophy of the nails (asymptomatic nail atrophy without skin or mucous membrane lesions) and 20-nail dystrophy (longitudinal striations; dull, rough surface, without permanent destruction of the nail) have been biopsied and proven to be lichen planus of 54, 98, Lichen planus of the nail in ~hi1dren.I~. the nails may be confused with psoriasis or onychomycosis. Mucosal involvement is very rare in children under 16 years: only a few cases of leukokeratotic oral mucous membrane lesions have been reported in the literature, as has a recent case of genital mucosal inv~lvement.~, 57, 84, lI4, lzo Erosive oral lichen planus has not been diagnosed in children under 16 years of age,15 but has been reported in one 16 year old.’lYThere is much debate in the oral surgery literature surrounding the potential for malignant transformation of oral lichen planus. The data range from 0.14% after 6 years to 2.5% after 3 years, all in older individua l ~ . Although ”~ oral lichen planus is not considered a premalignant lesion, there may be some risk to warrant follow-up, especially in the older population. Differential diagnosis includes aphthous ulcers, cicatricial pemphi-

Table 2. LICHEN PLANUS VARIANTS Variant Linear Annular Bullous Atrophic Hypertrophic

Morphology

Differential Diagnosis

Papules distributed in a zosteriform pattern along trunk or extremityS7,5 8 , 84 Papules in annular pattern on trunk, penjs57,58. 84 Vesicles or bullae surmounting pre-existing papules, or more rarely, de n0v043,68

Linear psoriasis, linear porokeratosis, inflammatory linear verrucous epidermal nevus57.58, 84 Granuloma annulare, sarcoid, tinea corporis, psoriasisS7.58. 84 Bullous impetigo Bullous erythema rnultiforme lrnrnunobullous 68 Extragenital lichen sclerosus, morphea Psoriasis57

Actinic

Few lesions, associated with atrophy Verrucous plaques, usually on pretibial legS7 Photodistributed’l’

Lichen planopilaris (follicular)

Follicular lesions with associated scarring alopecia18,84

Polyrnorphous light eruption, other photosensitivity”’ Pseudopeladel8.

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goid, and other blistering diseases with oral involvement.

may be resistant to conservative topical therapies and require more aggressive systemic treatment.

Treatment ROSACEA

Oral antihistamines (diphenhydramine, hydroxyzine) and topical corticosteroids are the mainstay of treatment. Medium potency topical corticosteroids are usually required (e.g., triamcinolone 0.1Yo); for hypertrophic lesions, more potent preparations may be needed (e.g., fluocinonide) with or without occlusion.8,l1 For extensive involvement, oral corticosteroids have been used in children (1 to 2 mg/kg/day for 1 to 2 week periods then weaned).8,49 Adverse effects of oral corticosteroids must be considered. Oral acitretin treatment (0.5 mg/kg/day for 12 weeks) of extensive, acute eruptive lichen planus in a 9 year old boy has been de~cribed.~ The risks of oral retinoids include premature epiphyseal closure in children41; however, recent reports of long-term use in children show that short term, low dosages (< 1 mg/kg/day) may be safe.94 Treatment of lichen planus of the nail can be difficult. Because the extent of involvement is variable, treatment has ranged from nothing to oral, intramuscular, and intralesional steroids. Application of potent topical steroids to the proximal nail fold is the least invasive treatment, though it may not be very efficacious. If the child has 20-nail dystrophy, which does not progress to permanent destruction, a conservative approach may be most appropriate. The risks of permanent scarring in all other forms of nail matrix lichen planus may justify the early use of oral p r e d n i ~ o n eAlthough .~~ intralesional steroids have been used successfully in adults, the procedure may be too painful and distressing for use in children. Other therapies of lichen planus in childhood include dapsone and griseofulvin (500 mg/day for 4 to 6 weeks in patients 10 to 55 years of age).57, 115 There is one report of PUVA treatment in an 8 year old boy93;however, the long-term risks of carcinogenesis should be kept in mind when considering PUVA treatment of a child. Lichen planus is a relatively rare disorder in children. Diagnosis may be difficult, especially if the presentation is atypical or isolated to the nail. It is important to recognize that lichen planus may have an atypical appearance in childhood, and that some variants

Rosacea is a chronic facial eruption that occurs primarily in middle-aged adults, peaking between the ages of 30 and 50 years. Rosacea is rare in children, but has been reported.19 Ocular rosacea (conjunctivitis, blepharitis, keratitis, chalazion) also peaks between the ages of 30 and 50 years, but has been described in ~hi1dren.l~. 21 Clinical Features

The clinical picture of rosacea in children resembles that seen in adults. There is a vascular component characterized by facial erythema, flushing, and telangiectases, and an inflammatory component, with papules and pustules located most commonly on cheeks, chin, and forehead (Fig. ,).I9 Rhinophyma has not been reported in children. Because of the acneiform appearance of the inflammatory lesions, the eruption may be mistaken for acne vulgaris. The presence of vascular lesions (e.g., telangiectases), as well as the absence of comedones, should point to a diagnosis of 10sacea.l~However, acne vulgaris and rosacea may coexist in some patients. Rosacea in children may also appear similar to perioral dermatitis, and to other more unusual papular facial eruptions like sarcoid and papular granuloma annulare. Erythematous facial eruptions such as polymorphous light eruption or the malar rash of systemic .lupus erythematosus can also be confused with 10sacea.I~Skin biopsy may be required to establish the diagnosis of rosacea, or to rule out these other entities. Perioral dermatitis is not rare in children.78 Papules and pustules are predominantly located around the nose and eyes as well as the There may be ill-defined erythema and fine scale (Fig. 7). There is no vascular component or ocular involvement; however, this eruption is often caused or exacerbated by the use of fluorinated topical steroids on the face.33 History will establish the cause, although topical steroid use can be occult (see Fig. 7). Granulomatous perioral dermatitis is a variant of perioral dermatitis in which small,

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Figure 6. Rosacea in an 13-year-old girl: dense red papules on cheeks, chin, and nose. A marked background of erythema was also present. Patient cleared after two months of oral tetracycline and topical metronidazole.

monomorphous, skin-colored papules develop in periorificial areas of the face, without erythema or scale.35,63 Alternate names suggested for this condition include granulomatous periorificial dermatitis and facial AfroCaribbean childhood eruption (FACE), reflecting its high incidence in black children.63, 134, 135 Histology may show superficial dermal and perifollicular granuloma^.^^,^ Because the clinical features of perioral dermatitis and rosacea sometimes overlap, and these conditions respond to the same therapy, some au-

thors suggest that granulomatous perioral dermatitis may be a variant of r ~ s a c e aPeri.~~ oral dermatitis may be a variant of rosacea as well. Treatment Therapy of rosacea in children is similar to that used in adults, and includes topical metronidazole gel and systemic antibi0ti~s.l~ Tetracycline should not be used in children

Figure 7. Perioral dermatitis in an 11-year-old girl resulting from prolonged use of mometasone cream that belonged to an aunt. Note perioral and perinasal distribution.

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under 9 years of age because of the risk of dental staining. For older children, tetracycline or doxycycline may be used in dosages similar to those used in adults. Erythromycin may be substituted for young children, or metronidazole gel alone can be used. Therapy is often required for a few months before tapering. Ocular rosacea can be treated with systemic or topical antibiotics and ophthalmic topical steroids.21 Perioral dermatitis and granulomatous perioral dermatitis are treated the same as rosacea in children, with topical metronidazole gel alone or in combination with systemic antibiotic^.^^, 63, 78, 83 The granulomatous variant may respond to treatment more slowly or not at all.35,44, 63 SWEET’S SYNDROME (ACUTE FEBRILE NEUTROPHILIC DERMATOSIS)

Sweet’s syndrome, or acute febrile neutrophilic dermatosis, is most commonly seen in women between the ages of 30 and 50 years. Since the initial series reported by Sweet in 1964, there have been numerous reports of Sweet’s syndrome in adults. About 10% of adults have an associated malignancy, such as acute myelocytic leukemia or preleukemia lZ3 syndrome.66, Sweet’s syndrome is rare in the pediatric age group. It has been reported in infants as young as 7 weeks, in children of all ages, and equally in both sexes.13, 61, 71 Sweet’s syndrome occurs in children without underlying disease, and in those with malignancies and infection^.^, 6 6 , 86 Malignancies reported in association with Sweet’s syndrome in children are similar to those seen in adults, and include acute myelogenous leukemia, juvenile chronic myelogenous leukemia, and myelodysplastic syndrome that progressed to acute myelocytic le~kemia.~,66 Associated infections include purulent rhinitis, otitis media, tonsillitis, osteomyelitis, pneumonia, and aseptic meningitis.13, 72, 75-77, 112 The etiology of Sweet’s syndrome is not known, but may involve a hypersensitivity reaction to an infectious or tumor antigen, or result from inap20, 38, 45 propriate cytokine ~ecretion.’~, The association of granulocyte colony stimulating factor (G-CSF) with Sweet’s syndrome has been documented in adults with underlying malignancy, and in a 5 year old child with glycogen storage disease Type Ib.37,38, 53, 97 717

Necrotizing vasculitis and pyoderma gangrenosum have also resulted from G-CSF therapy in G-CSF may cause Sweet’s and other neutrophilic dermatoses and vasculitides by stimulating neutrophilic function and release of ~ y t o k i n e s . ~ ~ Clinical Features

As in adults, Sweet’s syndrome in children is often preceded by an upper respiratory infection, and is associated with fever.20,45 There may be peripheral leukocytosis and an elevated erythrocyte sedimentation rate. The skin lesions are bright red tender plaques and nodules that are occasionally annular or bul1 0 ~ s .38,~61, . 71 They are usually multiple and are often located on the face, although they may develop on the trunk, extremities, or more rarely, palms, soles, and mucous membranes. Pathergy may occur.45Without treatment, skin lesions resolve after 1 to 6 months with no scarring; however, development of localized cutis laxa after Sweet’s syndrome is well documented in both children and adults, a phenomenon referred to as Marshall’s synd r ~ m e . ~ l70, , 79, 112 One child with cutis laxa and elastolysis after Sweet syndrome later developed fatal vascular Sweet’s syndrome is often mistaken for cellulitis, and patients may receive multiple courses of antibiotics before the diagnosis is made. Histopathology is diagnostic, and consists of a dense dermal infiltrate of mature neutrophils, and papillary dermal edema 66 Bullous Sweet’s must without vasc~litis.~~, be differentiated from erythema multiforme major and from immunobullous diseases such as chronic bullous disease of childhood. Treatment

Most patients with Sweet’s syndrome respond dramatically to systemic glucocorticosteroid therapy. Prednisone at 2 mg/kg/ day is initiated with slow tapering over a few months. Pediatric patients have been treated with alternatives such as colchicine and dapsone, but not with potassium iodide.4,13, 51 Recognition and treatment of any underlying infection or malignancy are also critical. MYCOSIS FUNGOIDES

Mycosis fungoides (MF) is most common in adults over 50 years of age, but can present

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in childhood. It may not be recognized for years or even decades, and as a result, children and adolescents with MF are often undiagnosed until adulthood.10,64, 138 Therefore, incidence figures for MF in childhood, which range from 0.5% to 4.3%, are probably underfound estimates.'", 64, 99, 138 Zackheim et that 24 of 557 patients (4.3%)with MF developed disease by age 20 years; a biopsy diagnosis was made by age 20 in only 13. The authors also described a man diagnosed at age 25 years who initially presented at age 22 months. At that time, the biopsy was thought to be consistent with parapsoriasis; however, when this initial biopsy was reviewed 23 years later, it was thought to be diagnostic of In young patients, the incidence of MF in males is equal to that in females. The prognosis and natural history of mycosis fungoides in young patients are probably similar to those in older patients, and depend on stage: those with earlier stage disease do well.'", 138 More advanced stages of MF have been reported in children: Sezary syndrome developed in an 11 year old girl with MEs1Serious long-term sequelae, such as the development of lymphoreticular malignancy, have also been documented: Hodgkin's disease developed 9 to 14 years after diagnosis of MF in 2 of 5 patients in whom MF was diagnosed before 20 years of age.99 Pityriasis lichenoides chronica, pityriasis lichenoides et varioliformis acuta, and follicular mucinosis can precede or occur in conjunction with MF in childhood and adolescence.lo,32, 64, 138 Follicular mucinosis is rare in children and is very rarely associated with MF in this age group. In contrast, coexistent MF is present in about 15% of adults with follicular mucinosis.lo,88 Distinct histopathologic features allowing differentiation of idiopathic alopecia mucinosa in childhood from that associated with MF include a lymphocytic infiltrate confined to follicular, perifollicular, or perivascular zones, a lack of Pautrier microabscesses, and the absence of plasma cell or eosinophil-containing inflammatory dermal infiltrate.88 Large plaque parapsoriasis is rare in children but may precede MF; some authors con64, 82, 99 The sider this entity equivalent to case of the 25 year old man described above by Zackheim et illustrates the diagnostic problems that can occur, as the initial biopsy was misinterpreted as parapso~iasis.'~~ Close follow-up is indicated in all children with

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large plaque parapsoriasis, with occasional skin biopsy to rule out MF. Clinical Features

The morphology of the skin lesions of MF in children resembles that seen in adults with similar stages of disease. For example, pediatric patients with patch stage MF present with indolent, asymptomatic, scaly patches and plaques. Because of this morphology, patch stage MF is often confused with nummular, contact, and atopic dermatitis, contributing to the difficulty in diagnosis. Variants such as p ~ i k i l o d e r m a ,discoid ~~ lesions with depressed centers and elevated borders,@localized swelling and scaling in digits,13hand macular hypopigmentation", lZ1, 132, 138 have been described in children. In particular, the hypopigmented variant is more common in younger patients, and predominantly in those with darker skin.69,121, 132, 138 In the series of Zackheim et al,13821% of patients with onset by 20 years presented with hypopigmentation. Hypopigmented MF presents as widely scattered, smooth or slightly scaly, hypopigmented patches that are usually asymptomatic (Fig. 8). The eruption may be mistaken for tinea versicolor, pityriasis alba, or vitiligo. lZ1,13*, 138 Skin biopsy is diagnostic.69, Treatment

The treatment of MF in pediatric patients is the same as that in adults, and depends on stage. Treatment alternatives for early MF include PUVA, UVB, potent topical steroids, and topical chemotherapy (carmustine, nitrogen mustard), while more advanced stages are treated with electron beam irradiation and systemic chemotherapy.'", 81, 99 H YPOPigmented MF may be treated with PUVA or UVB.69*132 In one study a child with plaquestage MF stage 11A was treated with PUVA plus alpha interferon.lz5 OTHER "ADULT" SKIN DISEASES lmmunobullous Diseases

Immunobullous diseases are rare in childhood and are often misdiagn~sed.'~~ These diseases are characterized by autoimmune reactions to antigens in the skin, and include

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Figure 8. Hypopigmented mycosis fungoides on the back of a 17-year-old boy who presented with generalized cutaneous disease, without lymph node or systemic involvement (stage T2). Scar over right scapula is from skin biopsy.

pemphigoid, pemphigus vulgaris and foliaceus, dermatitis herpetiformis, and epidermolysis bullosa acquisita. Most of the litera130 ture consists of individual case reports.lo3< Weston et aP30 found only 23 direct immunofluorescence (D1F)-proven cases of immunobullous disease in patients under 18 years of age out of 115 specimens in a single laboratory over a 16-year period.130 Misdiagnoses based on clinical findings were common, and included bacterial impetigo, poison oak dermatitis, Stevens-Johnson syndrome, and porphyria. Dermatitis herpetiformis was the most clinically overdiagnosed blistering disease. The authors emphasize the need to thoroughly evaluate children with suspected immunobullous disease with routine histology, direct IF, and serum for indirect IF. Negative IF should be repeated and the site of biopsy considered carefully (e.g., normal perilesional skin for DIF).130 Rabinowitz and Esterly103recently and comprehensively reviewed inflammatory bullous diseases in children. The authors stressed the rarity of these diseases in childhood, and the necessity of performing diagnostic skin biopsies and DIF in suspected cases.lo3 Lichen Sclerosus Lichen sclerosus (LS) is not rare in children: 10% to 15% of cases of LS occur in children, almost all girls. Extragenital LS also occurs in

children.80Familial LS has been reported in sisters, and in mother and daughter.l'O<118 LS in childhood is clinically and histologically identical to LS in adulthoods0;however, there are some issues of diagnosis and management of LS that are unique to the pediatric populalo5 LS in tion with this disease (Table 3).80, prepubertal boys is rare, but is probably underdiagnosed. It may present with phimosis.6, lo5,lZ6LS in girls, especially of early onset, resolves at menarche in up to half of cases, possibly because of alterations in hormone levelss0;however, even after active LS resolves, residual scarring and pigmentary changes may remain. In one series, signs of LS persisted in most girls, and the author disputes that resolution of LS at puberty is common or is hormonally related.lo5 Table 3. DISTINCTIVE FEATURES OF LS IN CHILDHOOD

-

Resolution at puberty in up to half of cases. May present with constipation due to pain on defecation, or with dysuria and secondary enuresis in girls. Topical testosterone contraindicated in prepubertal girls because of side effects of virilization. Differential diagnosis does not include premalignant vulvar dystrophies. May be confused with sexual abuse at presentation, especially if hemorrhagic or bullous features are present. Rarely, sexual abuse may result in worsening of LS.

ADULT SKIN DISEASE IN THE PEDIATRIC PATIENT

The differential diagnosis of LS in girls includes lichen simplex chronicus and rare entities such as immunobullous diseases and lichen planus. Premalignant vulvar dystrophy does not occur in childhood.'j LS may be misdiagnosed as genital trauma as a result of sexual abuse, especially when hemorrhage, bullae, and erosions are present6,80, 95; however, sexual abuse should be confirmed or ruled out in girls with worsening LS: a 7 year old girl experienced exacerbation of genital LS that was later attributed to sexual abuse.'O' The isomorphic response may play a role in worsening of LS in this setting. Vulvar changes caused by sexual abuse include hematomas, friability of the posterior fourchette, lacerations, and scarring from healed lacerations.y5 Potent topical steroids are effective in adult women with LS, and can be used in children.6, 16, '05 In women, topical testosterone is also frequently used; however, it is contraindicated in children because of side effects such 95, Topical progesterone or as virilizati~n.~~. estrogen in an emollient base has been used successfully in the treatment of LS in prepubertal girls.6,80, 95, 116 Combining topical steroids and topical progesterone therapy is another useful strategy.80

CONCLUSION

We have reviewed the distinctive features and management of selected "adult" skin diseases in children. However, it is critical that every dermatologist who sees children in practice recognizes the subtle differences in presentation of all skin disease in patients of this age group, and understands the important differences in management of cutaneous disorders in young patients.

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