[Urticarial vasculitis as a symptom of Muckle-Wells syndrome?].
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.
Grassegger
A
A
Universitätsklinik für Dermatologie und Venerologie.
Greil
R
R
Feichtinger
J
J
Fritsch
P
P
Hintner
H
H
D000328:Adult; D000686:Amyloidosis / Q000473:pathology*; D006801:Humans; D008297:Male; D010488:Polyarteritis Nodosa / Q000473:pathology*; D000685:Serum Amyloid A Protein / Q000032:analysis; D012867:Skin / Q000098:blood supply / Q000473:pathology; D012871:Skin Diseases / Q000473:pathology*; D013577:Syndrome; D014581:Urticaria / Q000473:pathology*
D000685:Serum Amyloid A Protein
Urtikarielle Vaskulitis als Symptom des Muckle-Wells-Syndroms?