Verplancke P, Driessen L, Wynants P, Naeyaert J M
Department of Dermatology, University Hospital Gent, Belgium.
Dermatology. 1998;196(4):463-6. doi: 10.1159/000017951.
The Schöpf-Schulz-Passarge syndrome is a rare genodermatosis with autosomal recessive transmission. It is characterized by palmoplantar keratoderma, eyelid apocrine hydrocystomas, hypodontia, hypotrichosis and hypoplastic nails. Several epithelial tumors have been described in this syndrome. This report describes a case with actinic keratoses, two tumors of the follicular infundibulum and one poroma with follicular differentiation. This is the first report of an association between the Schöpf-Schulz-Passarge syndrome and a poroma with follicular differentiation.
舍普夫-舒尔茨-帕萨热综合征是一种罕见的常染色体隐性遗传的遗传性皮肤病。其特征为掌跖角化病、眼睑顶泌汗腺汗管囊瘤、牙发育不全、毛发稀少和指甲发育不全。该综合征中已描述了几种上皮性肿瘤。本报告描述了一例患有光化性角化病、两个毛囊漏斗部肿瘤和一个具有毛囊分化的汗孔瘤的病例。这是舍普夫-舒尔茨-帕萨热综合征与具有毛囊分化的汗孔瘤之间关联的首次报告。