The Schöpf-Schulz-Passarge syndrome.

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.