A double-blind, randomized, placebo-controlled trial of topical tacrolimus 0·1% vs. clobetasol propionate 0·05% in childhood vitiligo.

BACKGROUND: Both clobetasol propionate 0·05% (CP 0·05%) and tacrolimus 0·1% (T 0·1%) ointments have been shown to be efficacious and safe in treating vitiligo in the paediatric population. OBJECTIVES: To assess efficacy and safety of these two therapies compared with each other and with placebo. METHODS: In this prospective study, children aged 2-16 years with vitiligo, stratified into 'facial' (n = 55) and 'nonfacial' (n = 45) groups, were randomized into three arms: CP 0·05% ointment (n = 30), T 0·1% ointment (n = 31) and placebo (n = 29) for 6 months. Successful repigmentation, defined as > 50% improvement, was evaluated by comparing photographs taken at baseline and at 2, 4 and 6 months. RESULTS: In the facial group, 58% of the CP 0·05% group responded successfully compared with 58% of the T 0·1% group, and in the nonfacial group, 39% of the CP 0·05% group responded compared with 23% of the T 0·1% group (P > 0·05). There was a significant difference in response between the CP 0·05% group vs. placebo (P < 0·0001) and the T 0·1% group vs. placebo (P = 0·0004). Spontaneous repigmentation was evaluated as 2·4%. No significant clinical adverse events were noted in any group. CONCLUSIONS: Both CP 0·05% and T 0·1% ointments offer similar benefit in paediatric vitiligo, both facial and nonfacial. The facial lesions responded faster than the nonfacial ones.

Localized scleroderma in childhood: a report of 30 cases.

Localized scleroderma (LS), a rare disease that occurs primarily in the pediatric age group, differs from systemic sclerosis (SSc) in that it is usually limited to the skin and subcutaneous tissue and is only rarely associated with systemic manifestations. The authors' experience with pediatric LS seen in 30 patients at a tertiary care center was reviewed: 26 had linear scleroderma, 19 on an extremity and 7 on the face; 3 had morphea; and 1 had generalized morphea. Antinuclear antibodies were present in 76% and rheumatoid factor in 39%. Five of 19 patients with linear scleroderma that involved an extremity had growth failure in that limb, and 1 required surgery. Sclerodermatous involvement over a joint resulted in limited range of movement in 6 patients, and 1 required surgery. One of the 30 patients developed SSc and polymyositis. There was difficulty in evaluating disease activity and hence in evaluating treatment. This experience with a large patient population suggests that LS, although usually a self-limiting disease, can result in significant morbidity.

Disseminated superficial actinic porokeratosis. Coexistence with other porokeratotic variants.

The coexistence of disseminated superficial actinic porokeratosis (DSAP) with other variants of porokeratosis is rare. We report three such cases: DSAP with porokeratosis of Mibelli; DSAP with linear porokeratosis; and DSAP occurring in the mother of a girl with linear porokeratosis. Although different areas of skin and different family members usually express the same morphological variant, we suggest that the simultaneous expression of two closely linked gene loci could explain the coexistence of different porokeratotic variants.

Neonatal lupus erythematosus.

Neonatal lupus is categorized by typical clinical features and the presence of maternal auto-antibodies. Mothers are uncommonly affected with clinical disease. The major clinical manifestations in neonates are cardiac, dermatological and hepatic, with rare instances of haemolytic anaemia and thrombocytopenia. The major morbidity and mortality result from complete congenital heart block. Dermatological manifestations occur mainly over the face and present with plaques of erythema with central atrophy, a mid-facial erythema, atrophy around the eyes and a telangiectatic variety. The long term outcome is usually excellent.

Localized morphea in children.

We are following 60 patients with morphea and/or linear scleroderma at the Hospital for Sick Children, Toronto. Lesions may vary in depth from epidermal changes resembling lichen sclerosis, to superficial and deep dermal changes. The subcutaneous tissue, bone and muscle may be involved. There is no good clinical marker of the disease. Because of the variation in the time to cure, it is difficult to evaluate treatment regimens. We are currently using methotrexate weekly either orally or subcutaneously and monthly pulsed steroids for three months.

Acne.

Acne is a disease that primarily affects adolescents but it may also be seen in neonates, children and adults. The main areas of involvement are the face, chest and back. Many individuals with acne are embarrassed by their condition, and even refuse to go to school and become socially withdrawn. Early treatment can prevent the devastating physical and emotional sequelae of acne. There are numerous effective therapies, and it is not necessary for patients to go through the trauma that plagued previous generations.

Localized cutaneous scleroderma.

Localized scleroderma in children includes morphea mainly on the trunk and linear scleroderma on the limbs, scalp, and face. Progressive systemic sclerosis is very rare in children and change from localized to progressive disease is extremely rare. Laboratory abnormalities occur with localized scleroderma, including eosinophilia, positive antinuclear factor (ANF), and increased immunoglobulin (Ig)G. The diseases are usually self limited, but involvement over bones may lead to marked functional impairment. Those on the face may be associated with underlying abnormalities and, occasionally, seizures. Treatment regimens are difficult to assess as there is no chemical marker, but penicillamine has gained wide use. It has numerous side effects and patients should be carefully monitored, particularly with regard to renal function. Other modalities have been used with variable success, but series of patients are small. Many medications produce skin softening in patients with progressive systemic sclerosis. The pathogenesis of skin sclerosis involves complicated interactions between vascular responses, lymphokines, and connective tissue proliferation. The etiology is completely unknown, despite a few cases associated with increased Borrelia titers.

Eczema.

Eczema is a term used to describe a variety of conditions which primarily include atopic dermatitis, seborrheic dermatitis, contact dermatitis, both irritant and allergic, scabies, tinea infections and immunodeficiencies. Some physicians use the term solely to describe atopic dermatitis. The article reviews clinical findings, etiology, diagnosis, prognosis, treatment, complications and new therapies for atopic dermatitis.

The use of topical steroids in children.

Topical steroids have been used with great success since the early 1950s. Knowledge of the mode of action, development of tests of potency, and manipulation of side chains have added to our understanding of these agents. Vehicles influence their rate of absorption. In the last three decades, topical and systemic side effects have been recognized with their inappropriate use. These side effects are seldom seen in children and should not preclude the correct use of topical steroids in inflammatory disorders of the skin, particularly atopic dermatitis.

Subcutaneous fat necrosis of the newborn: a review of 11 cases.

Subcutaneous fat necrosis (SFN) of the newborn is uncommon and usually occurs in the first weeks of life following a complicated delivery. The frequency with which hypercalcemia develops as a complication is uncertain. We report the clinical features of SFN in 11 patients seen between 1991 and 1998. Ten were born by cesarean section and fetal distress was present in the majority. It was not possible to distinguish SFN from sclerema neonatorum by time of onset or related infant diseases. Hypercalcemia developed in four infants up to 7 weeks after the onset of SFN. Infants with this condition should be carefully monitored for hypercalcemia.

New drugs in pediatric dermatology.

A number of important therapeutic agents have recently become available for the treatment of infectious and inflammatory skin diseases. Five of these drugs, calcipotriol, EMLA (eutectic mixture of local anesthetics), interferon-alpha 2a, cyclosporine, and acyclovir, are reviewed. Calcipotriol, a vitamin D analogue, has been shown to be useful but not curative in chronic stable plaque psoriasis in adults. Its use in children is being studied. EMLA is proving to be useful in the prevention of pain prior to minor procedures. The use of interferon-alpha 2a promises to be highly effective in the treatment of the complications of vascular lesions. Cyclosporine is a powerful immunomodulating agent. It has been used in the treatment of atopic dermatitis and psoriasis. Adverse effects limit its widespread use as a systemic agent. Topical cyclosporine has limited efficacy due to its poor penetration. Acyclovir has revolutionized the treatment of herpes simplex virus infections, particularly in reducing the morbidity and mortality of neonatal herpes. Whether it is indicated in varicella infection in normal children is questionable.

The diagnostic value of café-au-lait macules.

Single café-au-lait macules (CALMs) are common in the pediatric population and in most children represent a normal finding. It is important to recognize whether the presence of multiple CALMs in a particular patient is normal or indicates an association with a multisystem disorder. This article addresses issues concerning the prevalence, genetics, and natural history of CALMs in the general population and reviews disorders in which CALMs are present as a characteristic trait.

Ultrastructural features of epidermolysis bullosa.

Epidermolysis bullosa (EB), a heterogeneous group of hereditary diseases, varies in mode of inheritance, extent, severity, and presence or absence of scarring and dystrophy. Fourteen cases (13 in infants and 1 in a young adult) were studied. Subtyping by ultrastructural findings in normal and blistered skin biopsies was as follows: EB simplex (2), EB letalis (3), EBD dominant (2), and EBD recessive (7). One case diagnosed as recessive dystrophic by electron microscopy (EM) followed a benign course with little scaring and was reclassified clinically and after reviewing the EM as dominant dystrophic. Defining the level of bulla formation by EM allowed accurate diagnosis of subtypes. In 6 patients with EBD recessive, normal and bullous skin showed collagenolysis and no anchoring fibrils. In patients with EBD dominant, rudimentary fibrils were noted in normal skin. Whether absence of anchoring fibrils is primary or secondary in these two types and the role of collagenolysis remain unresolved.

Cutaneous mastocytosis in children: anaesthetic considerations.

Despite considerable information and discussion about the risk of serious complications in patients with systemic mastocytosis undergoing general anaesthesia, little is known specifically about the risk to patients with isolated cutaneous mastocytosis. The experience of 29 general anaesthetics in 12 children with urticaria pigmentosa and three with solitary cutaneous mastocytoma was reviewed. No major complications were encountered and the four minor problems seen were self-limiting. The data from this study do not suggest that patients with urticaria pigmentosa or solitary cutaneous mastocytoma are at increased risk of life-threatening complications under general anaesthesia.

Eosinophilic pustular folliculitis in infancy.

Five infants under 1 year of age were reported with a syndrome of recurrent crops of pruritic papulopustules of the scalp. In three children there were also intermittent outbreaks on the trunk and extremities. Cultures showed the pustules to be sterile. Biopsies of scalp and skin tissues showed eosinophilic folliculitis. Some patients had eosinophilia during outbreaks of pustules. These cases are similar to the eosinophilic pustular folliculitis reported in a few adult patients with the exception that there was predominant scalp involvement in the children. We propose that eosinophilic pustular folliculitis of infancy is a distinct pustular dermatosis.

Vulvitis circumscripta plasmacellularis mimicking child abuse.

Vulvitis circumscripta plasmacellularis (VCP) is a rare, benign vulvar disorder that is typically described in adult women. Our case occurred in an 8-year-old girl. The primary diagnostic concern was sexual abuse. VCP may also mimic lichen sclerosus, extramammary Paget's disease, pemphigus vulgaris, fixed drug eruption, squamous cell carcinoma, candidiasis, allergic contact dermatitis, and herpes simplex infection. Evaluation should include a biopsy because the histopathologic features of VCP are distinctive.