Treatment of Livedoid Vasculopathy with the Janus Kinase Inhibitors: A Case Series

Livedoid vasculopathy (LV) is a rare chronic recurrent occlusive disease characterized by painful ulcers and atrophic porcelain-white scars. Owing to the lack of strict treatment guidelines, the commonly used traditional treatment methods are not effective in some patients. Therefore, there is an urgent need to develop new therapies for LV.

Here we report 4 cases of LV that were successfully treated with the Janus kinase (JAK) inhibitors. The present study revealed a significant improvement in clinical activity and pain relief in patients with LV after the administration of abrocitinib (3 patients) or upadacitinib (1 patient). The median composite clinical score derived from erythema, ulceration, and physician-assessed pain fell from 7.25 ± 0.25 at baseline to 4.50 ± 0.50 at 4 weeks, 2.50 ± 0.50 at 8 weeks, and 1.00 ± 0.707 at 12 weeks. The remission time ranged from 5 to 8 weeks, with a mean remission time of 6.75 ± 1.258 weeks. Two patients experienced disease recurrence after the discontinuation of JAK inhibitor therapy. The average relapse time was 13.00 ± 7.071 months after drug withdrawal. The mean follow-up period was 6.00 ± 1.414 months. During the long follow-up period, only 1 patient showed a slight increase in the total cholesterol level, and no other adverse reactions were observed. Our study included 1 pediatric patient treated with upadacitinib. No adverse reactions occurred in this pediatric patient.

We believe that JAK inhibitors could be a promising therapeutic option for LV.