Original articleCongenital localized scleroderma
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
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Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis
2018, Pediatric Clinics of North AmericaCitation Excerpt :Gastrointestinal, pulmonary, cardiac, and renal problems are rare and not associated with evolution toward jSSc.13,33,37 LS can present at birth or in the toddler age as areas of faint erythema or violaceous color, or atrophy,38 with colored lesions mistaken for a nevus flammeus or port-wine stain19 (see Fig. 12B, D). Other patients present with extracutaneous involvement, such as arthritis, arthropathy, seizures, or headaches, which can precede the appearance of skin lesions by months to years.8,36
Scleroderma in children
2017, Best Practice and Research: Clinical RheumatologyCitation Excerpt :An epidemiological study in the United Kingdom reported an incidence rate of 3.4 cases per million children per year, the vast majority represented by the linear subtype [4]. The female-to-male ratio of JLS is 2.4:1 and the mean age at onset is approximately 7.3 years [49], although the disease can start as early as at birth as in congenital LS [50]. At this age, a misdiagnosis of skin infection, nevus, or salmon patch may lead to a consistent delay in diagnosis.
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2016, Handbook of Systemic Autoimmune DiseasesLocalized Scleroderma
2015, Textbook of Pediatric Rheumatology