Muckle-Wells syndrome: 4 cases in three generations.

Background. Muckle-Wells syndrome is a hereditary condition with variable p enetrance. The main manifestations are urticarial rash, malaise in the even ing, joint pain, perception deafness and renal amylosis. Case report. We describe a family with 4 affected members in 3 successive g enerations. Clinical expression was variable. Discussion. Despite the absence of renal amylosis in our patients,this fami ly presented the syndrome described by Muckle and Wells in 1962 As for othe r cases reported in the literature, the clinical course was Favorable with low-dose corticosteroid therapy.