[Multiple eccrine hydrocystomas of the eyelids in the framework of Schöpf syndrome. A case report].
Schöpf-Schultz-Passarge syndrome is a rare ectodermal dysplasia described by Schöpf in 1971. The disease is characterized by hypodontia, hypotrichosis, palmoplantar keratoderma, hypoplastic nails and eyelid hydrocystomas. We report the case of a 71-year-old man, the first French description of this syndrome, who remarkably presented all the signs. The patient also had skin tumors, multiple eccrime hidrocystomas and aspectific macular degeneration. A review of the literature is discussed.
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Clinique d'Ophtalmologie, HIA du Val de Grâce - Paris.
Dordain
M
M
Boucher
E
E
Metge
F
F
Millet
P
P
Maille
M
M
Maurin
J
J
D000368:Aged; D000848:Anodontia / Q000175:diagnosis*; D004439:Eccrine Glands / Q000473:pathology*; D005141:Eyelid Diseases / Q000175:diagnosis*; D006801:Humans; D007039:Hypotrichosis / Q000175:diagnosis*; D007645:Keratoderma, Palmoplantar / Q000175:diagnosis*; D008297:Male; D009260:Nail Diseases / Q000175:diagnosis; D012878:Skin Neoplasms / Q000175:diagnosis; D013577:Syndrome