ERYTHROKERATODERMIA VARIABILIS PRESENT AT BIRTH - CASE-REPORT AND REVIEW OF THE LITERATURE

A healthy boy had the distinctive lesions of erythrokeratodermia varia bilis (EKV) at birth. Twenty-eight patients described in the literatur e had EKV that presented in childhood. Nine of the 28 were said to hav e had a rash since birth, but none were distinctive of EKV. To our kno wledge this is the first well-documented case describing a child born with the skin manifestations of EKV. We conclude that patients with EK V are infrequently born with a rash, and that only very rarely when th e rash is present is it suggestive of the disorder.