[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.


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


CONCLUSIONS
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.
Buxtorf
K
K
Service de Dermatologie et Vénéréologie, Centre Hospitalier Universitaire Vaudois, CHUV/DHURDV, CH-1011 Lausanne, Suisse.
Cerottini
J P
JP
Fellrath
J M
JM
Debétaz
L F
LF
Guillod
J
J
Panizzon
R G
RG
D000293:Adolescent; D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis / Q000235:genetics; D018771:Arthralgia / Q000175:diagnosis / Q000235:genetics; D003638:Deafness / Q000175:diagnosis / Q000235:genetics; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis / Q000235:genetics; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012873:Skin Diseases, Genetic / Q000175:diagnosis / Q000235:genetics*; D013577:Syndrome; D014581:Urticaria / Q000175:diagnosis / Q000235:genetics*