YOU ARE NOW CONNECTED TO THE MEDLINE (1994 - 97) FILE. ==VASCULITIS== 1 AUTHOR Kumar L AUTHOR Thapa BR AUTHOR Sarkar B AUTHOR Walia BN TITLE Benign cutaneous polyarteritis nodosa in children below 10 years of age--a clinical experience. SOURCE Ann Rheum Dis 1995 Feb;54(2):134-6 ABSTRACT OBJECTIVE--To report 10 children younger than 10 years of age with benign cutaneous polyarteritis nodosa (BCPAN). METHODS--Ten children aged 1.25-10 years (mean 4.7 years; M:F = 7:3) were admitted with an unusual vasculitis. The clinical features, laboratory investigations, treatment and follow up data were analysed. RESULTS--Clinical features of these patients included: fever (10), peripheral gangrene (eight), livido reticularis (four), ulceration, nodules and vesiculobullous lesions alone or in combination (10), black necrotic patches over limbs and trunk (three), and arthralgia or swelling of large joints (seven). Cryoglobulinaemia was transient in three children, lasting for eight months in one of them. Histopathology of the skin lesions revealed vasculitis of small and occasionally medium sized blood vessels in nine of the 10 children. Possible association of BCPAN was noted with diphtheria-pertussistetanus immunisation (one), drugs (two), streptococcal infection (two), wasp sting (one), and falciparum malaria (one). The clinical course was interspersed with remissions and exacerbations. Response to corticosteroids alone occurred in seven patients, while three children needed cytotoxic drugs in addition. In a follow up of 5.6 years (mean) no evidence of systemic involvement was noted. CONCLUSIONS--A rare form of vasculitis, BCPAN, is reported in children. The features that distinguished our patients from those reported earlier were onset in the first decade of life and higher incidence of peripheral gangrene. YOU ARE NOW CONNECTED TO THE TOXLINE (1981 FORWARD, NON-ROYALTY) FILE. 1 AUTHOR O'Donnell B AUTHOR Black AK TITLE Urticarial vasculitis. SOURCE Int Angiol 1995 Jun;14(2):166-74 ABSTRACT Urticarial vasculitis describes a distinct entity in which the gross cutaneous lesions resemble urticaria and histologically show features of a vasculitis. The incidence of vasculitis in patients with apparent urticaria is between 2% and 20%. The diagnosis is suggested clinically by more persistent (lasting > 24 hours) and more symptomatic weals than in ordinary urticaria and by the presence of residual bruising. In addition to the skin the musculoskeletal, respiratory, renal and gastrointestinal systems may be involved in the disease, which is probably an immune complex mediated process. Urticarial vasculitis is most commonly an acquired idiopathic phenomenon but may occur in association with other disorders most often systemic lupus erythematosus, Sjogren's syndrome and serum sickness. In this article we review the background, histopathology, clinical features, extracutaneous manifestations, pathogenesis, aetiology, associated diseases, investigation, treatment, course and prognosis of urticarial vasculitis. 2 AUTHOR Gibson LE AUTHOR Su WP TITLE Cutaneous vasculitis. SOURCE Rheum Dis Clin North Am 1995 Nov;21(4):1097-113 ABSTRACT Cutaneous vasculitis comprises a wide spectrum of clinical syndromes and histopathologic findings that share the common theme of vascular inflammation and blood vessel damage. Clinical pattern and proper histopathologic studies are essential to the proper classification and evaluation of vasculitis. This article discusses the major types of cutaneous vasculitis typified by necrotizing or leukocytoclastic vasculitis and includes discussions of Henoch-Schonlein purpura, urticarial vasculitis, erythema elevatum diutinum, cutaneous polyarteritis nodosa, livedoid vasculitis, and cutaneous granulomatous vasculitis. 4 AUTHOR Mituiki K AUTHOR Hirakata H AUTHOR Oochi N AUTHOR Nagashima A AUTHOR Onoyama K AUTHOR Abe M AUTHOR Okuda S AUTHOR Fujishima M TITLE [Nephrotic syndrome due to membranous glomerulopathy in hypocomplementemic urticarial vasculitis syndrome;--a case report] SOURCE Nippon Jinzo Gakkai Shi 1994 Jul;36(7):863-70 ABSTRACT We report a case of hypocomplementemic urticarial vasculitis syndrome (HUVS) with membranous glomerulopathy in a 62-year-old man who had a 2-month history of secondary iritis. He was transferred to our hospital because of uncontrollable edema and respiratory dysfunction. Physical examination revealed anasarca, pulmonary edema, hypertension and urticaria-like eruption on his arms. Urinalysis, blood chemistry and serological studies showed massive proteinuria (10.5g/day) with numerous granular casts, hypoalbuminemia (1.5g/dl), renal dysfunction (creatinine; 1.6mg/dl, BUN; 86mg/dl), hypercholesterolemia (total cholesterol; 455mg/dl), positive results for antinuclear factor, microsome test, thyroid test, lupus anticoaglant, antithyroglobulin test and rheumatoid factor, but LE cell or double-strand anti DNA antibody was negative. Serum complement levels were persistently low as CH50 of 13 U/ml and Clq of 6.0 micrograms/dl. The patient serum precipitated with normal human Clq by immunodiffusion analysis, indicating the presence of anti-Clq antibody. Renal biopsy revealed membranous glomerulopathy with prominent fine granular deposition of Clq along the glomerular basement membrane by immunofluorescent study and subepithelial dense deposit by electron microscopy. Corticosteroid treatment was ineffective for hypocomplementemia and nephrotic syndrome. Acute subendocardial infarction occurred on the 25th hospital day and he died of acute respiratory distress syndrome on the 45th hospital day. Autopsy revealed leucocytoclastic vasculitis in the alveolar wall. HUVS was confirmed by clinical symptoms, such as iritis and urticaria-like eruption, serum anti-Clq antibody, the absence of any specific autoantibody for systemic lupus erythematosus (SLE) and leucocytoclastic vasculitis in the alveolar wall.(ABSTRACT TRUNCATED AT 250 WORDS) 1 AUTHOR Strickland DK AUTHOR Ware RE TITLE Urticarial vasculitis: an autoimmune disorder following therapy for Hodgkin's disease. SOURCE Med Pediatr Oncol 1995 Sep;25(3):208-12 ABSTRACT Immunological abnormalities have been described in patients with Hodgkin's disease, both associated with the malignancy itself and occurring secondary to therapy. These abnormalities often manifest as an immunodeficiency state, but can also present as immune dysregulation and autoimmune disease. We report two young patients with Hodgkin's disease who, following successful therapy, developed urticarial vasculitis (UV), a form of cutaneous autoimmune vasculitis. Both patients also had systemic symptoms including fever, an elevated erythrocyte sedimentation rate and serum copper, and abnormal in vitro studies of lymphocyte enumeration and proliferation. Distinguishing UV from recurrent Hodgkin's disease was especially difficult in one patient, and was possible only by lymph node biopsy. One patient has responded well to immunosuppressive therapy, while the other, who has more profound immune dysfunction, has developed a chronic autoimmune disorder. UV may thus occur in patients after therapy for Hodgkin's disease; we hypothesize that immune dysregulation, either associated with the malignancy or resulting from therapy, is important in the pathogenesis of this autoimmune process.