Efficacy of Tumor Necrosis Factor Inhibitors for Refractory Leg Ulcers in Cutaneous Polyarteritis Nodosa: A Case Series.

OBJECTIVE

Cutaneous polyarteritis nodosa (cPAN) is a rare necrotizing vasculitis that affects primarily small- to medium-sized arteries in subcutaneous tissue. cPAN is often characterized by a chronic and relapsing disease that presents with skin ulcers, livedo, and painful erythema. In this study, we evaluated the efficacy of tumor necrosis factor inhibitor (TNFi) treatment for cPAN-associated refractory leg ulcers.

METHODS

This retrospective study was conducted between 2016 and 2023 at 3 medical institutions in Japan and targeted patients with cPAN presenting with refractory leg ulcers who were treated with TNFi. The diagnosis of cPAN was histologically confirmed, and patients with secondary PAN were excluded. Data on the clinical background, treatment, ulcer status, and glucocorticoid (GC) dosage were collected, and the therapeutic efficacy of the treatment was evaluated.

RESULTS

Ten patients were included, with a mean age of 51 years, and 9 were female. All patients presented with recurrent leg ulcers. TNFi included adalimumab (5 cases), etanercept (4 cases), and infliximab (1 case). Complete epithelialization of the leg ulcers was achieved in all patients, and the average GC dose was successfully reduced from 20 mg/day to 3.5 mg/day. Additionally, 5 patients achieved a GC-free status. No serious adverse events were observed in any of the patients.

CONCLUSION

TNFi showed therapeutic efficacy for cPAN-associated refractory leg ulcers and enabled ulcer epithelialization and significant GC dose reduction. These findings support the utility of TNFi in the management of refractory leg ulcers in cPAN, highlighting the need for further large-scale studies to validate the results.