[Muckle-Wells syndrome: 4 cases in three generations].

BACKGROUND

Muckle-Wells syndrome is a hereditary condition with variable penetrance. The main manifestations are urticarial rash, malaise in the evening, joint pain, perception deafness and renal amylosis.

CASE REPORT

We describe a family with 4 affected members in 3 successive generations. Clinical expression was variable.

DISCUSSION

Despite the absence of renal amylosis in our patients, this family presented the syndrome described by Muckle and Wells in 1962. As for other cases reported in the literature, the clinical course was favorable with low-dose corticosteroid therapy.