Systemic lupus erythematosus with refractory ulcerated livedoid vasculopathy: Successful treatment with intravenous immunoglobulin and warfarin

Abstract

Key Clinical MessageWe reported a patient with systemic lupus erythematosus complicated by livedoid vasculopathy (LV), who responded well to intravenous immunoglobulin and warfarin. Cutaneous lesions of LV resemble those of cutaneous vasculitis. LV should be included in the differential diagnosis of leg ulcerations even in the presence of autoimmune disorders.

Efficacy and safety of intravenous immunoglobulin for treating refractory livedoid vasculopathy: a systematic review

“… 7 , 16 , 20 , 23 Eighty percent of pain was resolved after two IVIG cycles in a long-term follow-up study of 11 LV patients; 6 (2) ulceration healing usually lagged behind pain resolution. The resolution of skin ulcerations lasted from 1 to 14 cycles in the published studies; 6 , 7 , 8 , 12 , 14 , 16 , 19 – 23 (3) neurological symptoms such as numbness and hypoesthesia caused by ischemic nerve damage are common in LV. 24 However, conventional medications have little influence on LV neuropathy.…”

“…11 , 21 There were also a few LV patients who received IVIG as a single shot or at 2-week, 6-week, and 10-week intervals. 7 , 14 , 15 , 19 , 23 The interval for IVIG therapy was gradually prolonged on the basis of its efficacy and the patients’ response. The details in doses and duration of maintenance phase were limited and mainly depended on clinical experience of dermatologists and disease activity.…”

“…The details in doses and duration of maintenance phase were limited and mainly depended on clinical experience of dermatologists and disease activity. In maintenance phase, some LV patients paused IVIG therapy directly after receiving 1–3 cycles, 17 , 19 , 23 or gradually prolonged their intervals for IVIG infusions to 6-week, 8-week, or even 4- to 6-month interval. 5 , 7 Conventional treatments like anticoagulants or antiplatelet medications were gradually applied as combination therapy in maintenance phase.…”

Retiform non-blanchable purpuric plaques in a patient with systemic lupus erythematosus

“…Secondary APAS diagnosed (anti-phospholipid, anticardiolipin, antiphosphatidylserine, and antiphosphatidylinositol antibody tests were positive). Yoshioka et al 5 2018 Patient was positive for anti-dsDNA and ANA antibodies. Test was positive for anticardiolipin antibodies.…”

Intravenous immunoglobulin treatment: Where do dermatologists stand?

“…IVIG was used in the treatment of 10 patients with severe and resistant pyoderma gangrenosum who could not respond despite multidrug therapy, and improved in seven patients (Cummins, Anhalt, Monahan, & Meyerle, ). In treatment‐resistant livedoid vasculopathy and scleredema, it has been reported that IVIG is fast effective and safe treatment option (Kennemer, Pavlidakey, & Sami, ; Yoshioka, Tateishi, Kato, & Chen, ).…”