GVHD-Associated Angiomatosis

Graft-versus-host disease-associated angiomatosis (GVHD-AA) is a rare clinicopathologic variant of chronic GVHD characterized by benign vascular proliferations arising within areas of sclerodermatous skin change. The entity was formally named in 2014 and presents clinically as red-to-violaceous papules or nodules, confined to fibrotic GVHD skin. We present a case of GVHD-AA in a 29-year-old man with Philadelphia chromosome-positive acute lymphoblastic leukemia, diagnosed in 2017 and treated with matched unrelated donor allogeneic peripheral blood stem cell transplantation. His posttransplant course has been notable for severe chronic GVHD of the skin, eyes, mouth, and lungs, refractory to multiple systemic agents. His dermatologic symptoms began as sclerosis of the skin in the hands, arms, shoulders, and upper thighs, and have since progressed to involve 80% of his body surface area in 2025. Since 2023, he began developing numerous scaly red nodules overlying the scleroderma, which have been managed by shave removal, cauterization, and β-blocker therapy. Histological examination of shave biopsies from the arms and back shows an angiomatous proliferation with angiokeratoma-like features and Masson-like change. Endothelial cells are cytologically bland, and HHV8 immunohistochemistry is negative. This case adds to the limited body of histologically documented GVHD-AA and underscores the importance of recognizing this entity in sclerodermatous GVHD skin and relaying its prognosis and management to multidisciplinary clinical teams.

Abstract from PubMed