Acitretin in the treatment of erythrokeratodermia variabilis

A patient with erythrokeratodermia variabilis (Mendes da Costa's disease) is presented, and the clinical and histological response to acitretin is described. An initial dose of 35 mg of acitretin and a maintenance dose of 25-35 mg resulted in a pronounced and sustained improvement. Further reduction of the dosage resulted in a relapse within a few days. At the histological level the extensive hyperkeratosis and the moderate dermal inflammatory infiltrate decreased during treatment with acitretin. In comparison with the other retinoids available so far, acitretin is the derivative of first choice in the treatment of erythrokeratodermia variabilis Mendes da Costa.