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Multiple unilateral skin tumors suggest type 1 segmental manifestation of familial syringoma
European Journal of Dermatology. Volume 18, Number 3, 285-8, May-June 2008, Genes and skin
Free Article
Author(s) : Roeland PM Ceulen, Ariënne MW Van Marion, Peter M Steijlen, Jorge Frank, Pamela Poblete-Gutiérrez
Summary : An 18-year-old man presented multiple asymptomatic reddish-brown papules with a segmental distribution pattern confined to the left side of the trunk. These lesions had arisen two years before while the rest of the integument was unaffected. His further medical and family history was unremarkable. Histopathology revealed the characteristic features of syringoma. Since familial occurrence of syringoma with autosomal dominant inheritance has been described previously, we propose that the clinical phenotype observed in this patient reflects a type 1 segmental manifestation of familial syringoma and, thus, a cutaneous mosaicism.
Keywords : syringoma, familial syringoma, autosomal dominant inheritance, cutaneous mosaicism, segmental type 1 manifestation

Multiple unilateral skin tumors suggest type 1 segmental manifestation of familial syringoma