[Urticarial vasculitis as a symptom of Muckle-Wells syndrome?]

Hautarzt. 1991 Feb;42(2):116-9.
[Article in German]


A patient is reported with a history of several years of chronic urticaria, transient fever, arthralgias and secondary systemic amyloidosis. A biopsy of an urticarial lesion showed necrotizing vasculitis and amyloid deposits in the eccrine sweat glands. Amyloid A deposits were also detected in kidney and rectum biopsies. This patient is likely to represent a variant of the Muckle-Wells syndrome (chronic relapsing urticaria, fever, arthralgia, deafness and renal amyloidosis). Hitherto undescribed is the presence of a necrotizing vasculitis as cause of the urticarial rash; further investigation will determine whether or not this finding represents the rule rather than an exception.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Amyloidosis / pathology*
  • Humans
  • Male
  • Polyarteritis Nodosa / pathology*
  • Serum Amyloid A Protein / analysis
  • Skin / blood supply
  • Skin / pathology
  • Skin Diseases / pathology*
  • Syndrome
  • Urticaria / pathology*


  • Serum Amyloid A Protein