Abrocitinib for Refractory Livedoid Vasculopathy: A Case Report and Narrative Literature Review
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Xuchao Wang1, Qinglin Liu2, Shangshang Wang1
1Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, People's Republic of China.
Abstract:
Livedoid vasculopathy (LV) is a rare, chronic, pauci-inflammatory thrombo-occlusive microangiopathy of the dermal venules characterized by recurrent painful ulcers, livedo racemosa, purpura, and atrophie blanche. Conventional anticoagulant and antiplatelet therapy is frequently insufficient, and refractory disease remains a therapeutic challenge. We report a 48-year-old man with a 2-year history of painful, reticulated purpuric ulcers over the bilateral malleoli, histopathologically consistent with LV, who had failed sequential therapy with systemic corticosteroids, rivaroxaban, and compound rutin. Off-label treatment with abrocitinib 100 mg once daily was started as monotherapy, pain and exudation improved within 4 weeks, and by week 12 all ulcers had re-epithelialized, ankle edema and reticulated erythema had resolved, and inflammatory markers had normalized, without adverse events. The dose was tapered from month 3 and discontinued at month 6, and the patient remained free of recurrence during the 2-month post-discontinuation follow-up. A narrative literature search identified 18 reports and case series that together describe approximately 29 patients with LV treated with Janus kinase (JAK) inhibitors (baricitinib, tofacitinib, upadacitinib, or abrocitinib), most of whom improved. This hypothesis-generating observation is compatible with a therapeutic effect of abrocitinib in refractory LV, and it supports prospective evaluation of JAK inhibition in this disabling condition.
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