Erythrokeratodermia progressiva symmetrica

Abstract

10 cases of erythrokeratodermia progressiva symmetrica are described; 6 were familial, with autosomal-dominant inheritance. The disease started in early childhood, progressed during 1 or 2 years and then remained stationary with sharply marginated plaques remarkably symmetrical, usually sparing the thorax and abdomen. Histologically the picture was psoriasiform. The best therapeutic control was obtained with oral aromatic retinoids. The differential diagnosis is discussed.