Erythrokeratodermia variabilis in a Jewish Kurdish family

A Jewish family, originating from Kurdistan and presenting erythrokeratodermia variabilis in three consecutive generations, is described. The major features were the variable age of onset (from early infancy to 6 years) and the distinctive cutaneous lesions with demarcated erythematous hyperkeratotic plaques with irregular borders. The affected members had mild to severe expressions of the disease. The skin lesions were not influenced by puberty, pregnancy or old age. None of the patients had lesions of the palms and soles or abnormal neurological signs.