CLINICAL CASES   

Giornale Italiano di Dermatologia e Venereologia 2002 February;137(1):45-7

Copyright © 2002 EDIZIONI MINERVA MEDICA

language: Italian

Buschke-Ollendorf syndrome. A familial case

Gualandri L., Boccardi D., Ravanelli G., Menni S., Bernardo L.

Università degli Studi - Milano Clinica Dermatologica IV *Clinica Pediatrica Ospedale San Paolo - Milano

Buschke-Ollendorf syndrome is a rare autosomal dominant genodermatosis with full penetrance and variable expressivity. It is characterized by the presence of skin plaques and bone involvement with focal sclerotic patches in the epiphyseal and/or metaphyseal region of the long bones. In this report two members of the same family show a clinical picture suggestive for a diagnosis of Buschke-Ollendorf syndrome. Moreover, the main clinical features and different pathogenetical hypotheses about this syndrome are underlined.