Abstract

Livedoid vasculopathy (LV) is a rare thrombotic vasculopathy of the dermis characterized by painful, relapsing lower extremity ulcers. We performed a retrospective review of 53 cases of LV in an 8 year period at our institution. Diagnosis is challenging due to overlap in clinical appearance and nomenclature with other skin disorders. Ulceration is due to focal or multifocal occlusive thrombosis of dermal small vessels with ischemic changes related to vascular occlusion. Capillaries are mainly affected, followed by post-capillary venules and arterioles. The structure of vessel walls is preserved, though segments of vessel walls associated with thrombosis are thickened, with endothelial cell proliferation, mural fibrin and subintimal hyalinization. Intervening vessel segments are spared, which may cause findings to be missed on a single biopsy. There is only minimal lymphocytic inflammatory infiltrate; neutrophils and leukocytoclasis are not present, distinguishing LV from cutaneous vasculitis. Complement and immunoglobulin deposition have been noted in vessel walls by direct immunofluorescence but are non-specific findings. Thrombosis is typically limited to the superficial to mid dermis, only rarely occurring in subcutaneous fat. The epidermis is uninvolved except where there is ulceration or may be thinned in areas of post-ischemic scarring known as atrophie blanche. LV may be idiopathic or associated with hypercoagulable states, rheumatic disease, or venous stasis. The mechanisms that lead to thrombosis—particularly in the idiopathic form—are poorly understood. Histopathologic findings associated with LV should be noted in skin pathology reports along with co-observed alterations of venous stasis disease, if present. Variant pathology such as vasculitides and embolic occlusions should not be included under the nosology of LV.

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