Multiple unilateral skin tumors suggest type 1 segmental manifestation of familial syringoma.
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.
Ceulen
Roeland P M
RP
Department of Dermatology, University Hospital Maastricht, P. Debyelaan 25; PO box 5800, 6202 AZ Maastricht, The Netherlands. rce@sder.azm.nl
Van Marion
Ariënne M W
AM
Steijlen
Peter M
PM
Frank
Jorge
J
Poblete-Gutiérrez
Pamela
P
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D008875:Middle Aged; D009030:Mosaicism; D010641:Phenotype; D012867:Skin / Q000473:pathology; D012873:Skin Diseases, Genetic / Q000145:classification / Q000473:pathology*; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology; D018252:Syringoma / Q000235:genetics* / Q000473:pathology