[Rapp-Hodgkin's syndrome: two cases].

INTRODUCTION

In 1968, Rapp and Hodgkin described a family (a mother and her son and daughter) with a combination of dysplasic hair, nails, hypodontia and hypohidrosis associated with oral clefting and hypoplasia of the maxillary. Forty other cases of this congenital disorder have since been reported, and provide better knowledge of this predominantly hereditary disease.

CASE REPORT: We examined a 4 year-old boy for who had suffered since birth from a crusting and inflammatory scalp dermatitis which evolved periodically. He also had a coarse, wiry, light colored and slow growing hair, onychodysplasia, skin atrophy areas on the trunk, and distinctive craniofacial anomalies including a bifid uvula. He had never been seen to sweat. His father presented similar features, but less pronounced, and with a segmentary distribution.

DISCUSSION: We report two new cases of Rapp-Hodgkin's ectodermal dysplasia. We suspect a mosaicism in the father who may be the first and sporadic case of the family. These discret forms are frequently misdiagnosed and we hope we have provided some new elements which will help in the description of this rare disease. Early diagnosis permits an early follow-up and avoids numerous complications.