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

Ann Dermatol Venereol. 2000 Oct;127(10):822-4.
[Article in French]

Abstract

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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adolescent
  • Aged
  • Amyloidosis / diagnosis
  • Amyloidosis / genetics
  • Arthralgia / diagnosis
  • Arthralgia / genetics
  • Deafness / diagnosis
  • Deafness / genetics
  • Female
  • Humans
  • Kidney Diseases / diagnosis
  • Kidney Diseases / genetics
  • Male
  • Middle Aged
  • Pedigree
  • Skin Diseases, Genetic / diagnosis
  • Skin Diseases, Genetic / genetics*
  • Syndrome
  • Urticaria / diagnosis
  • Urticaria / genetics*