[Hair dysplasia in oculo-dento-digital syndrome. Apropos of a mother-daughter case]. / Dysplasie pilaire au cours du syndrome oculo-dento-digital. A propos d'un cas mère-fille.

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.

Multiple glomus tumors associated with multiple neoplasias.

We report the case of a 60-year-old man who presented with a lentigo maligna melanoma, which led to the discovery of a malignant endobronchial tumor and who had noticed the appearance of disseminated multiple glomus tumors about 10 years before. The late onset of this form of benign vascular tumor raises the question whether multiple glomus tumors may have been, in this case, a cutaneous marker of the development of underlying malignancy.

[Chronic urticarial eruption with arthritis, papillary edema, and meningoencephalitis of childhood onset]. / Eruption urticarienne chronique avec arthrites, cedème papillaire, méningo-encéphalite à début infantile.

The authors report on a case of chronic urticarial eruption since infancy in a man who is now 27-year-old; the association of numerous chronic symptoms, principally meningo-encephalitis, polyarthritis, hepato-spleno-adenomegaly allows the authors to compare this case to those described by Prieur and Griscelli in 1981 and 16 others cases found in literature. It appears as a "systemic disease" without biological or immunological markers; it might belong to the group of chronic normocomplementemic vasculitis.