- Mark DP Davis, MD
Mark DP Davis, MD
- Professor of Dermatology
- Mayo Clinic College of Medicine
- Section Editors
- Jeffrey Callen, MD, FACP, FAAD
Jeffrey Callen, MD, FACP, FAAD
- Editor-in-Chief — Dermatology
- Section Editor — Skin and Systemic Disease
- Professor of Medicine
- University of Louisville School of Medicine
- Eric L Matteson, MD, MPH
Eric L Matteson, MD, MPH
- Section Editor — Treatment Issues in Rheumatology
- Division of Rheumatology
- Professor of Medicine
- Mayo Clinic College of Medicine
- Deputy Editors
- Abena O Ofori, MD
Abena O Ofori, MD
- Deputy Editor — Dermatology
- Assistant Professor of Medicine, Dermatology Division
- Medical College of Georgia
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine, Part-time
- Harvard Medical School
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".)
The literature on livedoid vasculopathy is highly problematic with regard to nomenclature . 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).To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- EPIDEMIOLOGY AND PATHOGENESIS
- CLINICAL FEATURES
- ASSOCIATED DISORDERS
- DIFFERENTIAL DIAGNOSIS
- ADDITIONAL EVALUATION
- General measures
- Pharmacologic therapy
- - Our approach
- - Antiplatelet agents
- Aspirin with or without dipyridamole
- - Anticoagulants
- Warfarin or heparin
- Direct oral anticoagulants
- - Other therapies
- SUMMARY AND RECOMMENDATIONS