The hypocomplementemic urticarial-vasculitis syndrome: therapeutic response to hydroxychloroquine.
We report a patient with hypocomplementemic urticarial-vasculitis syndrome. This case illustrates the continuum between urticaria and purpura characteristic of hypocomplementemic urticarial-vasculitis syndrome. Clq precipitin was demonstrated in the patient's serum and in the diethylaminoethylcellulose-ion exchange fraction containing only IgG. A skin biopsy specimen of urticarial and purpuric lesions demonstrated leukocytoclastic vasculitis and granular deposition of C3 and Clq in the basement membrane with IgA, IgM, C3, and Clq in postcapillary venules. Serial total hemolytic complement activity and Clq determinations were performed, and the response to several treatment regimens is presented. Symptomatic and serologic improvement was observed only with hydroxychloroquine.
D000328:Adult; D001706:Biopsy; D003166:Complement Activating Enzymes / Q000032:analysis*; D015922:Complement C1q; D006462:Hemolytic Plaque Technique; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use*; D008297:Male; D012867:Skin / Q000473:pathology; D013577:Syndrome; D014581:Urticaria / Q000188:drug therapy / Q000276:immunology*; D014657:Vasculitis / Q000188:drug therapy / Q000276:immunology*
D006886:Hydroxychloroquine; D015922:Complement C1q; D003166:Complement Activating Enzymes