Buxtorf K, Cerottini J P, Fellrath J M, Debétaz L F, Guillod J, Panizzon R G
Service de Dermatologie et Vénéréologie, Centre Hospitalier Universitaire Vaudois, CHUV/DHURDV, CH-1011 Lausanne, Suisse.
Ann Dermatol Venereol. 2000 Oct;127(10):822-4.
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.
We describe a family with 4 affected members in 3 successive generations. Clinical expression was variable.
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.
