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 d
ermatosis from other childhood scaling erythemas. We present clinical,
histological and preliminary immunhistological data of a 4-year-old g
irl with the attributes of erythrokeratolysis hiemalis. No ancestors f
rom the endemic region were traced. The lack of further cases in the f
amily is interpreted as indicative of a spontaneous dominant new mutat
ion.