Livedoid vasculopathy in hemoglobinopathy-associated chronic leg ulcers

Ann Hematol. 2024 Dec 7. doi: 10.1007/s00277-024-06112-0. Online ahead of print.

Abstract

Chronic leg ulceration is a debilitating manifestation of hemoglobinopathies, and best management is uncertain. Livedoid vasculopathy (LV) is a cutaneous non-inflammatory thrombotic vasculopathy treated with anticoagulation that has been identified in hemoglobinopathy-associated chronic leg ulceration. However, most patients with hemoglobinopathy-associated ulcers do not undergo workup for secondary causes, and the prevalence and relevance of LV is unclear. Outcomes of secondary workup were examined retrospectively in this study. 108 patients with hemoglobinopathy-associated chronic leg ulcers were identified. 15% of patients underwent skin biopsy, and 97% of biopsies showed non-specific findings. Two patients had LV and neither responded to anticoagulants. Livedoid vasculopathy is a rare cause of ulceration in hemoglobin gene disorders and the benefit of anticoagulation in these cases is unclear.

Keywords: Leg ulcer; Livedoid vasculopathy; Sickle cell disease; Thalassemia; Thrombosis.