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Abstract

Background: Though not previously described, we have observed patients who have developed leukocytoclastic vasculitis (LCV) in the setting of chronic graft-versus-host disease (cGVHD). In this case series, we aimed to explore the relationship, if any, between these entities. Methods: Clinical, histopathologic, and direct immunofluorescence (DIF) data were extracted from the electronic medical record system for patients with biopsy-proven cutaneous LCV and concurrent cGVHD. Results: Four patients were included in the study, with a median (range) follow-up of 6 years (0.2-12 years). Clinically, all patients presented with palpable purpura, erythematous macules, or superficial ulcers on the lower extremities. Two patients developed vasculitic lesions overlying pre-existing sclerotic cGVHD on the lower legs. Histopathology in all four cases revealed small-vessel vasculitis with leukocytoclasis. One case also showed interface dermatitis. Dermal sclerosis was noted in two cases, suggestive of overlapping sclerotic cGVHD.  DIF testing showed granular IgA perivascular deposition (with other positive conjugates) in 2 cases and granular perivascular IgM and C3 in 3, and was negative in 1. In three cases, vasculitis onset followed tapering or discontinuation of systemic immunosuppressants, while one was presumptively triggered by acute renal injury. Corticosteroids led to resolution of LCV within 2–4 weeks, with 1 patient having a single recurrence. Conclusions: In patients who developed LCV in the setting of cGVHD, withdrawal of immunosuppression for GVHD prophylaxis may have been permissive. In this case series, vasculitis was limited to skin involvement and tended not to recur. Larger multicenter case series are required for further defining prognostic implications.