Hereditary cylindromatosis (HC; MIM 132700) is an autosomal dominant condit
ion characterized by benign skin appendage tumors most commonly on the scal
p and face. Previously, the HC gene (CYLD1) was linked to chromosome 16q12-
13, and tumors showed loss of heterozygosity (LOH), suggesting that CYLD1 i
s a tumor suppressor gene. Here we report a new multi-generation cylindroma
tosis family whose condition maps to that region, with 7/13 tumors showing
LOH on 16q.