[Follicular atrophoderma, basal cell proliferation and hypotrichosis (Bazex-Dupré-Christol syndrome). A study in 2 families]

Abstract

We describe a new case of Bazex, Dupré and Christol (BDC) syndrome in a family (family B...) where the disease affects four generations. Moreover, we report the clinical and genealogical evolution of the first family (family A...), described in 1966 by Bazex. A review of the literature has enabled us to collect the various symptoms of BDC syndrome, to discuss the relationship of this syndrome with basal cell naevomatosis and X-linked dominant chondrodysplasia punctata, and to discuss the kind of heredity. In family B..., two patients were examined. A one-month-old girl, with hypotrichosis and pseudo pili torti, and her 29-year-old father, with hypotrichosis and dorsal follicular atrophoderma of the hands. In this family, the paternal grand mother, one of her sisters, and the great grand father were also affected, but no basal cell carcinomas were found. In family A..., five patients were followed up. Four brothers, adult men aged 43 to 55 years initially presented only dorsal follicular atrophoderma the hands and face, hypotrichosis and numerous basal cell carcinomas of the face, especially in palpebral, temporal, nasal and forehead sites. Surgery was required and gave better results than radiotherapy, which left sites of sclerosis. Their mother was also affected and had been treated for numerous basal cell carcinomas of the face. The other members of the same generation (two women, two men) were unaffected. But the uncle and the grand father had the disease and at the last generation, a girl, child of one of the brothers affected, presented follicular atrophoderma and hypotrichosis.(ABSTRACT TRUNCATED AT 250 WORDS)