CHRONIC URTICARIA ASSOCIATED WITH ANGIONEUROTIC-EDEMA REVEALING FAMILIAL ALPHA-1-ANTITRYPSIN DEFICIENCY - EFFICACY OF DAPSONE

The authors report the case of a patient with chronic urticaria associ ated with angioneurotic oedema, in whom the aetiological assessment de monstrated serum alpha-1-antitrypsin deficiency. One of the patient's brothers had also suffered from angioneurotic oedema for several years and presented a low alpha-l-antitrypsin level. In our patient, combin ed treatment with H1 histamine receptor inhibitor and dapsone allowed control of the mucocutaneous manifestations. Dapsone, which has someti mes been tried successfully in panniculitis associated with alpha-1-an titrypsin deficiency, has a place in the therapeutic armamentarium for urticaria associated with alpha-1-antitrypsin deficiency and can be u sed after failure of conventional treatment.