PMID: 11908136Mar 23, 2002Paper

Schopf-Schulz-Passarge syndrome: 2 cases

Annales de dermatologie et de vénéréologie
T GkolfinopoulosC Blanchet-Bardon

Abstract

Schöpf-Schulz-Passarge's syndrome is a rare autosomal recessive genodermatosis associating hypodontia, palmoplantar keratoderma, cysts of the eyelid margins, onychodysplasia and hypotrichosis. We report two new cases. Case no. 1: A 49 year-old woman complained of erosive and fissured palmoplantar keratoderma. Nails were fragile and dystrophic. Permanent teeth were absent. She also had many small cysts of the eyelid margins and a middle hypotrichosis. There was no consanguinity between her parents. Case no. 2: A 56 year-old man was seen for red, scaly and well marked palmoplantar keratoderma. Permanent teeth were absent. He had a hair loss since the age of 30. Nails were hypoplastic and there were many small cysts of the eyelids. Biopsy of one of the cysts showed a follicular cyst associated with sweat duct dystrophy. Schöpf-Schulz-Passarge's syndrome was diagnosed in these 2 patients. There was no evidence of associated cutaneous tumors. Differential diagnosis of Schöpf-Schulz-Passarge syndrome include other genodermatoses comprising palmoplantar keratoderma and dental abnormalities. Benign or malignant tumors are frequently associated: eccrine poromas, eccrine syringofibroadenomas, follicular tumors, basal cell and squamous ce...Continue Reading

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