Livedoid vasculopathy in the Sri Lankan context – a case series

Abstract

Livedoid vasculopathy is a rare thrombo-occlusive vasculopathy marked by painful ulceration, livedoid changes, and skin atrophy with healing lesions, primarily affecting lower extremities. Diagnosis is often delayed due to variable presentation and overlapping features with autoimmune conditions. We present three histopathologically confirmed cases to illustrate the disease spectrum and different treatment options. This case series aims to identify the clinical features, serological profiles, different treatment options, and response in patients with livedoid vasculopathy. Three patients presenting to the rheumatology clinic at Teaching Hospital Peradeniya were evaluated for chronic recurrent ulceration in the ankle region. Clinical features, serology, and characteristic histopathology- luminal fibrinoid deposition, vessel-wall segmental hyalinization, and minimal perivascular inflammation were used to confirm the diagnosis of livedoid vasculopathy. Treatment was tailored based on patient-specific findings and included combinations of antiplatelets, immunosuppressants, corticosteroids, and vasodilators. Clinical response was monitored over time. One female and two male patients had chronic, recurrent painful leg ulcers healed with scarring (atrophie blanche). The female had recurrent leg ulcers for 6 years, positive antinuclear-antibodies (1:640 nuclear- homogeneous), non-scarring alopecia, inflammatory arthralgia; was diagnosed with Undifferentiated Connective Tissue Disease (lupus spectrum) and treated with tapering- steroids, aspirin, azathioprine, and hydroxychloroquine. One male had weakly positive lupus anticoagulant (confirmed on repeat testing), no autoimmune features and leg venous stasis risk factors; treated with tapering low-dose of steroids, aspirin, and compression stockings. The third had negative autoimmune markers and extensive ulceration; treated with tapering low-dose steroids, aspirin, bosentan. All responded with active ulcer healing and no recurrences. Livedoid vasculopathy is a rare and challenging disease with minimal evidence-based treatment options. The diagnosis should be considered in patients with recurrent, chronic ulcers. Bosentan is a newly attempted successful treatment option. Early diagnosis with histopathology and individualized management improves the outcome significantly.