Urticarial vasculitis as a symptom of Muckle-Wells syndrome?
Grassegger, A.; Greil, R.; Feichtinger, J.; Fritsch, P.; Hintner, H.
Der Hautarzt; Zeitschrift für Dermatologie Venerologie und verwandte Gebiete 42(2): 116-119
1991
ISSN/ISBN: 0017-8470 PMID: 1674741 Document Number: 379325
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.