Cutaneous leukocytoclastic vasculitis and myelodysplastic syndrome with little or no evidence of associated autoimmune disorders-a case report and a brief review of the literature.
Cutaneous leukocytoclastic vasculitis (CLV) is a necrotizing inflammatory disease of the small vessels in the dermis. Approximately 50% of cases with CLV are primary or idiopathic and the remainder may be associated with various diseases. Less than 1% CLV occurs in association with malignancies including leukemia, lymphoma, and myelodysplastic syndrome (MDS). The pathogenetic mechanism of CLV remains speculative and is generally believed to be related to autoimmune processes. We report here a 77-year-old white woman who presented with contemporaneous occurrence of CLV and MDS (WHO subtype RAEB-2). Autoantibodies and immune-complexes were not detected either in the serum or by direct immunofluorescense in the skin biopsy. The clinical course of MDS remained steady, but the cutaneous lesion resolved within 2 weeks with minimal or no specific therapy except corticosteroids. We suggest that absence of autoimmune phenomena and immune-complex in the skin lesions of CLV may be a hallmark of good prognosis.
DAS
Monisha
M
Department of Internal Medicine, University of Missouri-Kansas City, Kansas City, Missouri 64108-2741, USA. dasmo@umkc.edu
Chhabra
Rajiv
R
Hinton
Stuart W
SW
D000368:Aged; D000936:Antigen-Antibody Complex / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D001706:Biopsy; D005260:Female; D006801:Humans; D009190:Myelodysplastic Syndromes / Q000175:diagnosis*; D011379:Prognosis; D012867:Skin / Q000473:pathology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000175:diagnosis*
D000936:Antigen-Antibody Complex; D001323:Autoantibodies