Beijing Da Xue Xue Bao Yi Xue Ban. 2026 Oct 18;58(5):1081-1083.
ABSTRACT
Livedoid vasculopathy (LV) is a painful and thrombotic obliterative vascular disorder characterized by spontaneous thrombus formation within skin capillaries or small veins, ultimately leading to localized hypoxia and the development of skin ulcers. LV is a rare condition predominantly affecting young women, with an incidence rate of approximately 1/100 000. Clinically, it manifests as recurrent reticular green spots and painful ulcers on the distal lower extremities, which subsequently heal, leaving behind white and atrophic scars, termed "white atrophy". The skin manifestations of the disease are prominent, often seen in dermatology. Recently, the Department of Rheumatology and Immunology of Honghui Hospital, Xi ' an Jiaotong University, admitted a patient with LV. This young female patient presented with recurrent lower limb skin purpura and ulcers, which had occurred spontaneously during the summer for the past three years. Following a skin biopsy conducted at another hospital, she was diagnosed with LV and subsequently treated with phototherapy, anticoagulation therapy, and topical corticosteroids, yet there was no improvement in her condition. In April of this year, she sought further treatment at our hospital and was admitted for comprehensive care. After completing a thorough battery of relevant examinations, we excluded other potential vascular disorders, abnormal coagulation profiles, and autoimmune diseases, such as cutaneous polyarteritis nodosa (cPAN), leukocyte-cracking vasculitis (LCV), and antiphospholipid syndrome (APS). The patient remained definitively diagnosed with LV. Under our department's care, she received oral glucocorticoids, tacrolimus for immune modulation, and colchicine for anti-inflammatory effects. Subsequently, her symptoms improved, and she was discharged from the hospital. Four months post-discharge, a follow-up examination revealed significant improvement in her symptoms. The therapeutic approaches for LV encompassed general therapy, antiplatelet and anticoagulant therapy, glucocorticoid and immunosuppressant therapy, intravenous human immunoglobulin therapy, and hyperbaric oxygen therapy. Additionally, the use of biologics, including tumor necrosis factor inhibitors and small molecule targeted JAK inhibitors, had been reported in LV cases. Further randomized controlled trials are necessary to validate these treatment options. This report presented a case of LV where favorable outcomes were achieved through a combination of anticoagulant and immunosuppressive therapies. Nevertheless, given that the patient has been afflicted with the disease for three years and we have only conducted a four-month follow-up, which is relatively short, continued long-term follow-up of this patient remains imperative.
PMID:42880932