[Schopf-Schulz-Passarge syndrome: 2 cases].
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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 cell carcinomas. Tumors usually appear after the age of 60. Regular follow-up and biopsy of the suspect lesions are necessary.
Gkolfinopoulos
T
T
Service de Dermatologie I, Hôpital Saint-Louis, 1, avenue Claude Vellefaux, 75475 Paris.
Ingen-Housz-Oro
S
S
Cavelier-Balloy
B
B
Blanchet-Bardon
C
C
D001706:Biopsy; D002869:Chromosome Aberrations; D003560:Cysts / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D003937:Diagnosis, Differential; D005141:Eyelid Diseases / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D005143:Eyelids / Q000473:pathology; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009264:Nails, Malformed / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D012867:Skin / Q000473:pathology; D012873:Skin Diseases, Genetic / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D013577:Syndrome