HAIR SHAFT DYSPLASIA IN OCULO-DENTO-DIGIT AL SYNDROME - REPORT OF A MOTHER-DAUGHTER CASE

Oculo-dento-digital syndrome (SODD) as defined by Meyer-Schwickerath i n 1957 is a rare entity (84 cases) which belongs to ectodermal dysplas ias. It consists of: the characteristic features (long face, pinched n ose); syndactyly; ocular, dental and bone abnormalities. This entity i s usually transmitted on the autosomal dominant mode. We report two ca ses (a mother and daughter) with polymalformations which we classed as SODD. Furthermore, agenesis of lacrimal duct and genitourinary abnorm alities were noted. The mother had a very particular complex hair shaf t dysplasia (incomplete pili torti, ''tiger tail'' aspect, fractures) with alopecia since she was fifteen years old. The daughter's hair was normal at birth. In SODD, fine and sparse hair is often observed (44 p. 100). Only one patient had hair shaft investigation under polarized light : pili annulati and monilethrix were described but not found in our cases. These two reports incite to the systematic hair shaft stud y in SODD as other ectodermal dysplasia syndromes.