Schopf-Schulz-Passarge syndrome

Schopf-Schulz-Passarge syndrome

A rare autosomal recessive ectodermal dysplasia and genodermatosis (OMIM:224750) characterised by cysts and apocrine hidrocystomas of the eyelid margins, palmoplantar keratoderma, hypodontia, hypotrichosis, nail dystrophy and skin appendage tumours (e.g., eccrine syringofibroadenoma).
Molecular pathology
Defects in WNT10A cause Schopf-Schulz-Passarge syndrome.