A rash in a hairy situation: Leukocytoclastic vasculitis at presentation of hairy cell Leukemia

Abstract

A 62-year-old woman presented with a three-month history of fatigue, fevers, bruising, epistaxis, and rash. Physical examination revealed erythematous and violaceous non-blanching papules and macules, coalescing into patches and plaques on the bilateral lower and upper extremities, abdomen, and back (See Image 1 Panel A and B). Physical examination revealed bilateral supraclavicular lymphadenopathy and hepatosplenomegaly. Laboratory studies revealed pancytopenia. Bone marrow biopsy revealed hypercellular marrow with “fried egg” appearance, and immunophenotyping positive for CD11c, CD25, and CD103, diagnostic for hairy cell leukemia. A skin lesion biopsy revealed leukocytoclasis with extravasated erythrocytes on H&E (See Image 1 Panel C), as well as direct immunofluorescence positive staining for perivascular IgA, IgG, IgM, C3, and fibrinogen (See Image 1 Panel D), consistent with leukocytoclastic vasculitis. She was treated with topical triamcinolone and seven -day induction therapy with cladribine. Complete resolution of rash and leukemia occurred rapidly with sustained remission 14 months later. Malignancy is frequently diagnosed concurrent to autoimmune diseases including vasculitis.1 Vasculitis occurs simultaneously in 5% to 8% of primary lymphoproliferative disorders.2 This occurrence has been previously hypothesized to be autoimmune in nature due to cross-reactivity between hairy cells and vascular endothelium.3 Case reports have similarly shown resolution of symptoms after treatment of primary underlying hematologic malignancy.4, 5 Therefore, hematologic malignancies should be considered as a potential underlying etiology for newly diagnosed leukocytoclastic vasculitis. The authors declare no potential conflicts of interest.