Livedoid vasculopathy_0313 Derm Dx
A fifty-year-old female with no significant past medical history presents complaining of purplish lesions on her bilateral medial ankles. The lesions are painful and have ulcerated.
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Livedoid vasculopathy, has many synonyms, but it is most commonly also referred to as atrophie blanche or segmental hyalinazing vasculitis. Technically it is a vasculopathy and not a vasculitis.
In vasculitis the primary process is inflammation, which causes damage to the blood vessel walls. In a vasculopathy the primary problem is thrombosis within the vessel wall.
In both vasculitis and vasculopathy there can ultimately be both thrombosis and inflammation. It is important to understand whether the primary inciting event is a vasculopathic or a vasculitic process for diagnosis and treatment.
Clinically, in livedoid vasculopathy there are painful purpuric lesions that ulcerate and heal with small white stellate scars. This scarring is often referred to as atrophie blanche. The scars are frequently surrounded by a brownish hyperpigmentation. In most patients the lesions occur bilaterally.
Livedoid vasculopathy primarily affects females. The mean age of onset is 45 years. On biopsy, livedoid vasculopathy shows thrombi within blood vessels of the dermis. There may be evidence of hemorrhage and subsequent hemosiderin deposition in the dermis surrounding the affected vessels. This accounts for the brownish color that is seen in the skin surrounding the ulcer.
Many cases of livedoid vasculopathy are associated with venous stasis disease, and therefore the biopsy may also demonstrate lobular vascular proliferation — a hallmark of venous stasis.
Livedoid vasculopathy is due to a hypercoagulable state that causes thrombosis of the superficial blood vessels. This thrombus within the vessel causes tissue hypoxia and subsequent tissue damage.
With current testing modalities, a hypercoagulable state is only detected in approximately 40% of patients. The workup includes tests for anticardiolipid antibody, lupus anticoagulants, deficiencies in protein C or S, abnormalities for antithrombin III, and other available tests for hypercoagulable disorders.
Diagnosis & Treatment
Diagnosis is made by the clinical appearance of atrophic whitish reticulated scarring of the leg. Biopsy shows a hyalinizing vasculopathy.
When a specific underlying hypercoagulable state is identified, it must be treated and managed. Smoking cessation is imperative. Patients with underlying venous stasis disease may improve simply by management with elevation and compression. However, some of these patients may need medical therapy for livedoid vasculopathy.
The most commonly used medical therapy is low-dose aspirin. Other therapies that may be beneficial in select populations include but are not limited to: pentoxifylline, oral dipyrimadole, danazol, heparin and warfarin.
Adam Rees, MD, is a graduate of the University of California Los Angeles School of Medicine and a resident in the Department of Dermatology at Baylor College of Medicine in Houston.
- Bolognia J, Jorizzo JL, Rapini RP. ” Chapter 24: Cutaneous Manifestations of Microvascular Occlusion Syndromes.” Dermatology. St. Louis: Mosby/Elsevier, 2008.
- James WD, Berger TD, Elston DM et al. “Chapter 35: Cutaneous Vascular Diseases.” Andrews’ Diseases of the Skin: Clinical Dermatology. Philadelphia: Saunders Elsevier, 2006.