Progressive symmetric erythro-keratosis associated with oligodontia, severe caries, disturbed hair growth and ectopic nail: a new syndrome?

A 7-year-old girl had well-demarcated erythematous plaques covered with white pityriasiform scales which were symmetrically distributed and involved the extensor surfaces of the extremities as well as the abdomen, buttocks and face. Histological examination showed marked hyperkeratosis with parakeratosis, and a thickened granular cell layer, mild acanthosis and slight lymphocytic infiltration surrounding the papillary blood vessels, compatible with a diagnosis of progressive symmetrical erythro-keratosis. Remarkably, a keratotic excrescence similar to a normal nail plate involved the tip of the nose since the age of 6 months. Moreover, occipital hairlessness, oligodontia and severe caries were noted. Progressive symmetric erythro-keratosis has so far been described as a non-syndromic skin disorder, which is why our patient's multisystem birth defect may represent a new entity. 2008 S. Karger AG, Basel.