John Libbey Eurotext

European Journal of Dermatology

Multiple unilateral skin tumors suggest type 1 segmental manifestation of familial syringoma Volume 18, numéro 3, May-June 2008

Auteurs
Department of Dermatology, University Hospital Maastricht, P. Debyelaan 25; PO box 5800, 6202 AZ Maastricht, The Netherlands, Department of Pathology, University Hospital Maastricht, Maastricht, The Netherlands, Department of Maastricht University Center for Molecular Dermatology (MUCMD), University Hospital Maastricht, Maastricht, The Netherlands
  • Mots-clés : syringoma, familial syringoma, autosomal dominant inheritance, cutaneous mosaicism, segmental type 1 manifestation
  • DOI : 10.1684/ejd.2008.0389
  • Page(s) : 285-8
  • Année de parution : 2008

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.