A case of Schöpf-Schulz-Passarge syndrome.
Schöpf-Schulz-Passarge syndrome (SSPS) is a rare ectodermal dysplasia characterized by hypodontia, hypotrichosis, nail dystrophy, palmoplantar keratoderma, and periocular and eyelid margin apocrine hidrocystomas. Several other skin tumours have been described in association with this syndrome, in particular, multiple palmoplantar eccrine syringofibroadenoma (ESFA). We report a case of SSPS with diffuse palmoplantar hyperkeratosis, which was shown by histology and immunocytochemistry to be due to ESFA.
Hampton
P J
PJ
Pathology, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, UK. hamptonpj@doctors.org.uk
Angus
B
B
Carmichael
A J
AJ
D000368:Aged; D003560:Cysts / Q000473:pathology; D004476:Ectodermal Dysplasia / Q000473:pathology*; D005141:Eyelid Diseases / Q000473:pathology; D018251:Hidrocystoma / Q000473:pathology; D006801:Humans; D007039:Hypotrichosis / Q000473:pathology; D007645:Keratoderma, Palmoplantar / Q000473:pathology*; D008297:Male; D009260:Nail Diseases / Q000473:pathology; D013544:Sweat Gland Neoplasms / Q000473:pathology; D013577:Syndrome