title: [Hair dysplasia in oculo-dento-digital syndrome. Apropos of a mother-daughter case].
abstract: Oculo-dento-digital syndrome (SODD) as defined by Meyer-Schwickerath in 1957 is a rare entity (84 cases) which belongs to ectodermal dysplasias. It consists of: the characteristic features (long face, pinched nose); syndactyly; ocular, dental and bone abnormalities. This entity is usually transmitted on the autosomal dominant mode. We report two cases (a mother and daughter) with polymalformations which we classed as SODD. Furthermore, agenesis of lacrimal duct and genitourinary abnormalities were noted. The mother had a very particular complex hair shaft 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 study in SODD as other ectodermal dysplasia syndromes.
authors.affiliation: Service de Dermatologie, CHU Pontchaillou, Rennes.
authors.forename: H
authors.initials: H
authors.lastname: Adamski
authors.forename: J
authors.initials: J
authors.lastname: Chevrant-Breton
authors.forename: S
authors.initials: S
authors.lastname: Odent
authors.forename: M
authors.initials: M
authors.lastname: Patoux-Pibouin
authors.forename: B
authors.initials: B
authors.lastname: Le Marec
authors.forename: A
authors.initials: A
authors.lastname: Laudren
authors.forename: M
authors.initials: M
authors.lastname: Urvoy
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D005124:Eye Abnormalities / Q000150:complications; D005260:Female; D005385:Fingers / Q000002:abnormalities; D006197:Hair / Q000473:pathology / Q000648:ultrastructure*; D006201:Hair Diseases / Q000150:complications / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D018126:Odontodysplasia / Q000150:complications / Q000235:genetics*; D013576:Syndactyly / Q000150:complications / Q000235:genetics*; D013577:Syndrome; D014034:Toes / Q000002:abnormalities
vernacular_title: Dysplasie pilaire au cours du syndrome oculo-dento-digital. A propos d'un cas mère-fille.