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.
References in periodicals archive ?
Though the most common presentation is a solitary lesion, some cases of multiple apocrine hidrocystomas have been described often in association with Gorlin-Goltz and Schopf-Schulz-Passarge syndromes [4].