URTICARIAL VASCULITIS - AN AUTOIMMUNE DISORDER FOLLOWING THERAPY FOR HODGKINS-DISEASE

Immunological abnormalities have been described in patients with Hodgk in's disease, both associated with the malignancy itself and occurring secondary to therapy. These abnormalities often manifest as an immuno deficiency state, but can also present as immune dysregulation and aut oimmune disease. We report two young patients with Hodgkin's disease w ho, following successful therapy, developed urticarial vasculitis (UV) , a form of cutaneous autoimmune vasculitis. Both patients also had sy stemic symptoms including fever, an elevated erythrocyte sedimentation rate and serum copper, and abnormal in vitro studies lymphocyte enume ration and proliferation. Distinguishing UV from recurrent Hodgkin's d isease was especially difficult in one patient, and was possible only by lymph node biopsy. One patient has responded well to immunosuppress ive therapy, while the other, who has more profound immune dysfunction , has developed a chronic autoimmune disorder. UV may thus occur in pa tients after therapy for Hodgkin's disease; we hypothesize that immune dysregulation, either associated with the malignancy or resulting fro m therapy, is important in the pathogenesis of this autoimmune process . (C) 1995 Wiley-Liss, Inc.