ERYTHROKERATOLYSIS HIEMALIS - SCALING ERYTHEMATOUS GENODERMATOSIS WITH SEASONAL MANIFESTATION

Erythrokeratolysis hiemalis, keratolytic winter erythema or Oudtshoorn skin has been reported from the South African district of Oudtshoorn as a dominantly inherited dermatosis beginning in early childhood, in some cases with circinar scaling erythemas. Seasonal manifestation in wintertime and a characteristic multiform histology distinguish this dermatosis from other childhood scaling erythemas. We present clinical, histological and preliminary immunhistological data of a 4-year-old girl with the attributes of erythrokeratolysis hiemalis. No ancestors from the endemic region were traced. The lack of further cases in the family is interpreted as indicative of a spontaneous dominant new mutation.