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.

Tuberous sclerosis: clinicopathologic features and review of the literature.

INTRODUCTION: Tuberous sclerosis is a hamartoneoplastic syndrome, which may involve multiple organ systems. Oral hard tissue manifestations of the syndrome have been described in the literature only as recently as 1955. Patients who presented with clinical manifestations of tuberous sclerosis did not routinely undergo oral surveys to rule out 'lesions', and consequently data on 'lesions' in the maxillofacial complex is scant. Ten cases have been found in the English language literature, which describe maxillofacial 'lesions', which may be tumours, new growths, neoplasms or overgrowths occurring in patients diagnosed with tuberous sclerosis. PURPOSE: To review the literature for all maxillofacial lesions associated with tuberous sclerosis and to present an eleventh case of a patient with a maxillofacial lesion diagnosed as having tuberous sclerosis. RESULTS: Eleven cases were found with maxillofacial fibroblastic lesions associated with tuberous sclerosis. These lesions were all fibrous benign neoplasms found in the maxillofacial bony complex. CONCLUSIONS: Maxillofacial fibroblastic lesions in tuberous sclerosis have various histopathological presentations, some of which may be difficult to differentiate. Consequently, close microscopic examination of these lesions is necessary so that adequate surgical treatment is provided.

Inflammatory linear verrucous epidermal nevus and arthritis: a new association.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare, chronic skin condition that begins in early childhood. We present two children with ILVEN and arthritis, a previously undescribed association. We discuss the relevance of this association and suggest appropriate management for this arthritis.

Children with morphea have normal self-perception.

We evaluated the self-esteem and quality of life of 47 children with morphea with the use of the Harter self-perception profile for children and Visual Analog Scale. Most children with morphea have normal self-worth and a high quality of life. Morphea, like some other childhood chronic illnesses, does not impair self-esteem.

The health of mothers of children with cutaneous neonatal lupus erythematosus differs from that of mothers of children with congenital heart block.

PURPOSE: Neonatal lupus erythematosus is caused by the transplacental passage of maternal autoantibodies. The aim of this study was to determine the risk of connective tissue disorders in mothers of children with cutaneous neonatal lupus erythematosus, as compared with the risk in mothers of children with congenital heart block, which is also often caused by maternal autoantibodies. SUBJECTS AND METHODS: We prospectively studied all mothers of children with cutaneous neonatal lupus erythematosus during a 14-year period at the Hospital for Sick Children, Toronto, Ontario, Canada. We identified 28 mothers, of whom 24 were eligible for study. The health and antibody status of the mothers were determined at the birth of the child and at followup. RESULTS: All mothers had anti-Ro antibodies at the time of birth. Initially 10 mothers were healthy and 14 mothers had either a defined (n = 9) or an undifferentiated (n = 5) autoimmune disorder. At a mean follow-up of 7 years, 13 (1 of whom had died) had a defined connective tissue disease, and 5 had an undifferentiated autoimmune disorder. Only 6 (25%) remained asymptomatic. By comparison, 36 (56%) of 64 mothers of children with congenital heart block were asymptomatic at follow-up (P <0.005). CONCLUSIONS: The majority of mothers of children with cutaneous neonatal lupus erythematosus had a defined or undifferentiated autoimmune disorder at the time of the child's birth, and others developed these conditions during follow-up. The health of these mothers appears to differ from that of mothers of children with congenital heart block.

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.

Methotrexate and corticosteroid therapy for pediatric localized scleroderma.

INTRODUCTION: Localized scleroderma (LS) can cause permanent disability, and there is no universally accepted effective treatment. Methotrexate (MTX) has been shown to be effective in the treatment of systemic sclerosis. OBJECTIVES: To determine the efficacy and tolerability of MTX and corticosteroid therapy in patients with LS. METHODS: MTX, 0.3 to 0.6 mg/kg per week, was given to 10 patients (6 girls, 4 boys; mean age, 6.8 years; mean disease duration before starting treatment, 4 years) with active LS. In addition, pulse intravenous methylprednisolone, 30 mg/kg for 3 days monthly for 3 months, was given to 9 patients at the initiation of therapy. RESULTS: One patient discontinued taking MTX after a month; the remaining 9 patients responded. The median time to response was 3 months (95% CI, 1.15-4.85). One responder discontinued taking MTX after a year because of leukopenia; the LS worsened within 2 months. In another patient LS flared up after 10 months and responded to an increased dose of MTX and intravenous methylprednisolone. At the last follow-up visit, all patients who continued to receive MTX therapy had inactive skin lesions. CONCLUSION: Treatment with MTX and corticosteroids appears to be effective in the treatment of LS and is generally well tolerated. A placebo-controlled study is necessary to confirm the efficacy of MTX therapy in LS.

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.

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.

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.

An open study of tinea capitis in 50 children treated with a 2-week course of oral terbinafine.

BACKGROUND: Terbinafine is used in the treatment of dermatophyte infections. There have been several studies suggesting a good response to terbinafine in treating tinea capitis, specifically with dermatophytes of the Trichophyton species. METHODS: We enrolled 50 consecutive children with a clinical diagnosis of tinea capitis into an open study using terbinafine for 2 weeks. RESULTS: Clinical and mycologic cure occurred in more than 86% of patients with no side effects and good compliance. CONCLUSION: In this study terbinafine was a safe and effective treatment of tinea capitis in children, particularly when caused by the Trichophyton species.

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.

Erosive adenomatosis of the nipple in an eight-year-old girl.

Erosive adenomatosis of the nipple (EAN) is a rare, benign neoplasm of breast lactiferous ducts. Peak incidence is in the fifth decade in women. Clinically, it may be mistaken for Paget's disease and, histologically, for adenocarcinoma. Some authors have proposed an association with breast carcinoma and fibrocystic breast changes. Erosive adenomatosis of the nipple is extremely rare in children. Treatment is usually local excision of the nipple. This is the first formal English language case report of EAN in the pediatric age group. It is important for dermatologists to be familiar with this entity to prevent unwarranted breast removal.

Fixed drug eruptions in children.

To determine the anatomic location and offending drug in fixed drug eruptions (FDE) in children, we performed a 5-year retrospective analysis. Thirty-five children with FDE were evaluated. The most common cause of FDE was the combination drug trimethoprim-sulfamethoxazole.

Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin 1, in patients with localized scleroderma.

OBJECTIVE: Serum autoantibodies to fibrillin 1, the major component of microfibrils in the extracellular matrix, recently have been reported to occur in the tight skin mouse and in patients with systemic sclerosis, but not in patients with other connective tissue diseases. This study was undertaken to determine whether antifibrillin 1 antibodies could be detected in patients with localized forms of scleroderma. METHODS: Sera from 50 patients with localized scleroderma (27 with linear scleroderma and 23 with morphea) and 51 normal controls were tested for IgG and IgM antifibrillin 1 autoantibodies, using a radioimmunoassay (RIA) and a human recombinant fibrillin 1 protein (rFbn-1). RESULTS: Both in patients with linear scleroderma and in those with morphea, mean levels of IgM and IgG binding to rFbn-1 were significantly higher than in controls. Eight patients with linear scleroderma (30%) and 6 patients with morphea (26%) had IgG autoantibodies to fibrillin 1 (rFbn-1) by RIA, compared with 3 controls (6%) (P = 0.006 and P = 0.022, respectively). No correlations between antifibrillin 1 antibodies and active skin disease or antinuclear antibody positivity were found. CONCLUSION: Autoantibodies to fibrillin 1 occur in patients with both forms of localized scleroderma (linear scleroderma and morphea). The clinical and pathogenetic significance of this autoimmune response remains to be determined.

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.