Original article
Congenital localized scleroderma

https://doi.org/10.1016/j.jpeds.2006.04.052Get rights and content

Objectives

Juvenile localized scleroderma (JLS) usually has its onset during later childhood. This report describes the clinical and serologic features of six children with congenital localized scleroderma (CLS).

Study design

A large, multinational study was conducted among pediatric rheumatology and dermatology centers by collecting information on demographics, family history, triggering environmental factors, clinical features, laboratory reports, and treatment of patients with JLS. Patients with onset at birth were carefully examined.

Results

Among 750 patients with JLS, 6 patients (0.8%) had scleroderma-related lesions at birth. Female-to-male ratio was 2:1. All patients had linear scleroderma, in four involving the face with en coup de sabre appearance. Two patients were misdiagnosed as having skin infection, one nevus, one salmon patch, and two undefined skin lesions. The mean diagnostic delay was 3.9 years. In comparison with the group of 733 patients with late-onset JLS, CLS presented a significantly more prolonged disease duration at diagnosis and a higher frequency of en coup de sabre subtypes.

Conclusions

Congenital localized scleroderma is a rare and probably underestimated condition in neonates. The linear subtype was the exclusive manifestation of the disease. CLS should be included in the differential diagnosis of infants with cutaneous erythematous fibrotic lesions to avoid functional and aesthetic sequelae and to allow prompt therapy.

Section snippets

Study Design and Patient Selection

A large data collection including information on demographics, epidemiologic and clinical features, laboratory reports, and treatment of children with JLS was performed among 270 pediatric rheumatology and dermatology centers in Europe (n = 166), North America (n = 42), South America (n = 28), Asia (n = 30), Australia (n = 2) and Africa (n = 2).

The charts of 750 patients who met the criteria for the JLS diagnosis2 and disease onset by 16 years of age were reviewed. Patients with the onset of

Demographics and Subtypes

During the period January 2002 to June 2003, 70 centers (38 European, 12 North American, 11 South American, 8 Asian, and 1 Australian) took part in a multinational survey and reported 750 patients with onset of localized scleroderma by 16 years of age. Six children with scleroderma-related skin lesions at birth (0.8%) were reported, and data were analyzed. Their clinical characteristics are summarized in the Table.

At birth, the skin lesions were described by the parents as erythematous and

Discussion

Localized scleroderma, although uncommon, is a much more frequent type of scleroderma in childhood than is systemic sclerosis.1 However, to our knowledge, congenital onset has never been reported. We have described a series of six patients with onset of scleroderma-related findings at birth. Previous reported studies3, 4, 5, 6, 7 and one recent large, multicenter study8 show that JLS affects children during later childhood, with a mean age at disease onset ranging from 6.8 to 7.9 years, and

References (14)

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