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Livedoid vasculopathy

Author
Mark DP Davis, MD
Section Editors
Jeffrey Callen, MD, FACP, FAAD
Eric L Matteson, MD, MPH
Deputy Editors
Abena O Ofori, MD
Monica Ramirez Curtis, MD, MPH

INTRODUCTION

Livedoid vasculopathy is a chronic, painful, thrombo-occlusive cutaneous vasculopathy that involves the distal lower extremities and feet. Characteristic clinical features include livedoid skin changes (linear or angular, erythematous nodules), atrophie blanche (smooth, ivory-white plaques), and ulceration. The diagnosis is confirmed through a skin biopsy that demonstrates characteristic vascular abnormalities, including intraluminal thrombosis, endothelial proliferation, and subintimal hyaline degeneration.

The pathogenesis of livedoid vasculopathy is unclear. The disorder can occur either independently or in association with acquired or inherited thrombophilia or various systemic diseases.

The clinical manifestations, diagnosis, and treatment of livedoid vasculopathy are reviewed here. Cutaneous vasculitis and other causes of leg ulcers are reviewed separately. (See "Overview of cutaneous small vessel vasculitis" and "Approach to the differential diagnosis of leg ulcers".)

NOMENCLATURE

The literature on livedoid vasculopathy is highly problematic with regard to nomenclature [1]. A variety of other terms have been used to refer to this condition, most often "atrophie blanche" and "livedoid (or livedo) vasculitis." However, "atrophie blanche" is most appropriately used as a descriptive term for the smooth, ivory-white plaques that characteristically occur in livedoid vasculopathy but may also occur in other disorders, particularly venous insufficiency. The use of "vasculitis" should be avoided because true vasculitis, as demonstrated by destructive inflammation within the blood vessel wall, is absent. (See 'Clinical features' below and 'Pathology' below.)

Less common terms that have been used to refer to livedoid vasculopathy in the literature include segmental hyalinizing vasculopathy, livedo reticularis with summer ulcerations, livedo reticularis with winter ulcerations, and painful purpuric ulcers with reticular patterning on the lower extremities (PURPLE).

                     
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Literature review current through: Sep 2017. | This topic last updated: Sep 07, 2017.
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