Track: Case Reports

Abstract:

Graft-versus-host disease–associated angiomatosis (GVHD-AA) is a rare vascular proliferation arising in fibrotic skin of chronic GVHD. Lesions present as variably sized red to violaceous soft papules and nodules, some with erosion and significant adherent sanguinous crust, overlying areas of sclerosis. We report a case in a 29-year-old man with Philadelphia chromosome–positive acute lymphoblastic leukemia treated with matched unrelated donor peripheral blood stem cell transplantation in 2017. His course was complicated by severe chronic GVHD of the skin, eyes, mouth, and lungs, refractory to multiple systemic agents, with sclerosis involving 80% of body surface area. Beginning in 2023, numerous scaly red nodules developed overlying sclerodermatous skin. Shave biopsy specimens from the arms and back revealed a well-circumscribed angiomatous proliferation with angiokeratoma-like features and Masson change. Endothelial cells were cytologically bland, and HHV8 immunohistochemistry was negative. The skin lesions have been managed with shave removal, cautery, and propranolol without complete response. GVHD-AA is an uncommon clinicopathologic entity that may mimic malignant vascular neoplasms. Greater recognition of this overlooked vascular complication can prevent misdiagnosis as malignancy and ensure appropriate management in chronic GVHD.

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Michael Stephens