Venous ulcers in Behçet syndrome

Abstract

Introduction

Post-thrombotic syndrome (PTS) and stasis ulcers are late complications of deep vein thrombosis (DVT) in Behçet's syndrome (BS). We aimed to determine the clinical and histopathological characteristics, treatment modalities, and outcomes in BS patients with stasis ulcers.

Method

We included 63 BS patients with stasis ulcers from a total of 310 with vascular involvement, seen at a multidisciplinary center between January 2021 and July 2022. Data on demographics, clinical features, histopathology, radiology, and treatments were collected. Ulcer size, location, duration, and healing time were defined.

Results

Patients’ median age was 45 years, and age at vascular onset was 27 years. Except for 4 pts with only venous insufficiency, all had lower extremity DVT. Ulcers appeared a median of 3 years after vascular involvement onset and in 44 % healed imminently in a median of 6 months. At the time of evaluation in the current study, of the 63 patients with history venous ulcers, 35 (56 %) presented with active ulcers while the remaining presented with complete recovery of at least one-year duration. There were in total 202 ulcers with median ulcer size of 3 cm. 72 % were localized in the gaiter region. Histopathological examination was available for 21 pts. In 67 % (14/21), the diagnosis favored stasis dermatitis. No frank vasculitis was observed. Treatment included bed rest, local treatments, venous compression and immunosuppression. Patients received a combination of immunosuppressive agents, including biological DMARDs (75 %), non-biological DMARDs (97 %), and steroids (94 %). Despite these intensive therapies, ulcers remained unhealed in 17 %, and the recurrence rate was 73 % over a median follow-up of 16.8 years.

Conclusion

Leg ulcers are challenging complications of DVT in BS and represent an unmet medical need. Future studies should investigate the effectiveness of early immunosuppressive therapy, and other interventions in preventing venous ulcers and improving outcome.