Vasculitis with urticaria, hypocomplementemia, and multiple system involvement.
A patient with cutaneous necrotizing vasculitis had chronic urticaria associated with multiple system involvement including arthralgias, glomerulonephritis, myositis, pseudotumor cerebri, and adenopathy. Persistent hypocomplementemia is noted with classic pathway activation. The syndrome recognized in this patient and those few individuals reported previously seems to constitute a distinct category of collagen-vascular disease.
D000328:Adult; D002908:Chronic Disease; D003095:Collagen Diseases; D003165:Complement System Proteins / Q000172:deficiency*; D003937:Diagnosis, Differential; D005260:Female; D005921:Glomerulonephritis / Q000473:pathology; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis; D007249:Inflammation; D007592:Joint Diseases / Q000473:pathology; D008206:Lymphatic Diseases / Q000473:pathology; D009220:Myositis / Q000473:pathology; D011559:Pseudotumor Cerebri / Q000473:pathology; D012877:Skin Manifestations; D013577:Syndrome; D014581:Urticaria / Q000150:complications*; D014652:Vascular Diseases; D014680:Veins / Q000473:pathology*
D003165:Complement System Proteins