[Cowden disease: treatment with acitretine].

INTRODUCTION: Cowden's disease is a rare hereditary skin disease which is important to recognize due to the risk of cancer.

CASE REPORT: A 40-year-old man was hospitalized for psoriasis. He presented florid papillomatous lesions involving the lip and the buccal cavity, a thyroid adenoma and polyposis of the gut. The diagnosis of Cowden's disease was retained. Treatment with acitretin, 0.75 mg/kg/d was initiated for the psoriatic lesions. Hypertrophic lesions of the lip and mouth regressed during treatment but reappeared when the drug dose was tapered of then withdrawn. The course of the polyposis was not verified.

DISCUSSION: This case demonstrates the frequency of digestive tract involvement in Cowden's disease and the favorable, though transient, effect of oral retinoids on mucosal lesions in Cowden's disease.