A case of livedoid vasculopathy in a 22-year-old man

Journal of Drugs in Dermatology, Nov-Dec, 2004 by Aimee Leonard, Miriam Keltz Pomeranz, Andrew G. Franks, Jr.

Abstract

A 23-year-old man presented with a 3-year history of painful ulcerations and retiform scarring of his lower extremities. Skin biopsy revealed evidence of thrombotic vasculopathy. Livedoid vasculopathy is an uncommon condition presenting as painful lower extremity ulceration and scarring in the form of irregular, ivory-white, stellate plaques. Its pathogenesis is unclear, but it is thought to result from coagulation and fibrinolytic disorders causing occlusion of dermal vessels.

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Case Report

A 22-year-old man presented to the dermatology clinic at the Bellevue Hospital Medical Center in August 2003 for evaluation of a 3-year history of recurrent debilitating ulcers and scarring plaques of his dorsal feet and ankles. The lesions were extremely painful, with associated sensations of paresthesia. He was taking oral tramadol for severe pain associated with his condition. The patient had no other medical problems and had no systemic complaints. He denied a history of hepatitis or blood transfusion. His history was positive for tobacco use. He had been treated in the past with multiple courses of antibiotics, systemic corticosteroids, and pentoxifylline, which he discontinued after failing to see improvement. He was treated with aspirin and nifedipine with modest improvement in his symptoms.

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On physical exam, hyperpigmented, fibrotic plaques with retiform crusted erosions and white atrophic scars in a stellate pattern were present on the dorsal feet and ankles. The lesions were exquisitely tender to palpation. There were regions of crusting that expressed scant amounts of purulent drainage with pressure.

Complete blood count, basic metabolic panel, coagulation panel, urinalysis, erythrocyte sedimentation rate, hepatitis serologies, protein C, protein S, anti-cardiolipin antibody, lupus anti-coagulant, and rheumatoid factor were within normal limits. Serum protein electrophoresis revealed diffuse hypergammaglobulinemia with no abnormal band. C-reactive protein was 9.62 mg/L, fibrinogen was 608 mg/dL, and wound culture grew enterobacter aerogenes and pseudomonas aeroginosa.

A punch biopsy was performed, and hematoxylin and eosin stained sections show thrombotic material within small blood vessels in the deep dermis and subcutis consistent with thrombotic vasculopathy. There was sparse perivascular inflammation without evidence of vasculitis.

Discussion

Livedoid vasculopathy, or atrophie blanche, is an uncommon condition resulting in painful lower extremity ulceration and scarring. Women are affected more often than men, with a ratio of 4:1. The lesions can occur at any age, but the peak incidence ranges from 30-60 years (1). There does not appear to be a racial or geographic predilection.

This condition presents as purpuric macules and papules that progress to painful, irregular ulcers of the lower legs and dorsal feet. These ulcerations are often recurrent and chronic with spontaneous remissions and exacerbations that may be seasonal (2). They often progress to scarring with firm, ivory-white stellate plaques. Atrophie blanche and livedoid vasculopathy are terms that historically have been used interchangeably. However, it is important to recognize that "atrophie blanche" is a morphological term which describes the ivory-white plaques in this condition, but can be seen even in common entities such as chronic venous stasis without the preceding constellation of recurrent purpura and ulceration.

The pathogenesis of livedoid vasculopathy remains unclear, but it is believed to result from coagulation and fibrinolytic disorders causing occlusion of dermal vessels. Although it is not thought to have systemic manifestations, it may be seen in association with systemic lupus erythematosus, scleroderma, and arteriosclerosis (1). Livedoid vasculopathy is divided into a primary, or idiopathic type whose pathogenesis is unknown (2) and a secondary form, associated with an underlying immunologic or vasoocclusive condition (1). The disease has been observed in patients with factor V Leiden mutation, antiphospholipid antibody syndrome, hyperhomocysteinemia, protein C deficiency, and increased platelet activation (3).

Histopathology examination demonstrates a vasculopathy with endothelial proliferation, and dermal vessel occlusion with fibrinoid material or erythrocytes. The small number of polymorphonuclear leukocytes and the lack of nuclear fragments around the small vessels are the most important signs differentiating atrophie blanche from leukocytoclastic vasculitis (1).

Treatment of livedoid vasculopathy is challenging, as no single form of therapy has been consistently effective in ameliorating the disease. Modalities are often aimed at antiplatelet, anticoagulant and fibrinolytic effects (4). Therapies with demonstrated benefit include aspirin, coumadin, dipyridamole (5), low molecular weight heparin (6), pentoxifylline (7), enoxaparin (3), nifedipine (8), and tissue plasminogen activator (9). In addition, tobacco cessation should be advised.