SAT0551 Leg ulcers in behcet’s syndrome: an observational survey in 24 patients
Authors: Y. Ozguler, Z. Kutlubay, A.S. Dikici, M. Melikoglu, C. Mat, H. Yazici, E. Seyahi
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Formal experience with leg ulcers in Behçet's syndrome (BS) is limited. It is a rare complication that can be seen during the course of mainly pts with the post-thrombotic syndrome. They can be difficult to manage and may cause disability leading to unemployment and severe impairment in quality of life. <h3>Objectives</h3> In this observational survey, we aimed to describe clinical characteristics of pts with leg ulcers. <h3>Methods</h3> A total of 24 pts (23 M/1 F) that were seen at our multidisciplinary BS clinic between May 2016-Jan 2018 were evaluated with the help of a standardised questionnaire. Venous Doppler US and if necessary abdominal CT were used to evaluate localization of venous involvement. Biopsies were done if needed. Medical and other interventional treatments were recorded. <h3>Results</h3> The mean age at disease onset was 27.5±7.1 years. The median duration between the disease onset and ulcer development was 4.0 [2.5–11.5] years. The median follow-up was 7.8 [IQR: 2.9–14.2] years. Eleven (46%) were unemployed due to the leg ulcer. Venous involvement was present in 20 pts (83%). Lower extremity vein thrombosis was shown in all these 20 pts. It was mostly bilateral (15/20). Femoral veins were the most commonly involved (18/20), followed by popliteal veins (14/20), v. saphena magna or v. saphena parva (7/20), iliac veins or inferior vena cava (8/20). The same 9/20 pts had other large vessel involvement such as pulmonary artery (n=5) or dural sinus thrombosis (n=3). Four pts did not have any venous thrombosis or insufficiency. Histopathologic studies could be done in 3 and showed necrotizing vasculitis in 2 and venous stasis in the 3rd. Twelve pts (50%) had solitary ulcers and 12 had 2 or more. We observed a total of 34 ulcers in 24 pts. They were mostly found around the medial malleolus (15/34) and anterior surface of the tibia (14/34). Five pts had leg ulcers at unusual places such as lateral malleolus (n=2), popliteal fossa (n=1) and posterior surface of the tibia (n=2). Immunosuppressive agents including azathioprine, cyclophosphamide, interferon alpha, infliximab, and corticosteroids were used. Bed rest was advised to almost all. Iloprost infusions were given in 13 (54%) for a median duration of 6 mo. Additionally, larvae of Lucilia sericata were tried in 9 pts. Skin graft insertion was used in 2 pts, however, was successful in 1. We observed that in 11 (46%) pts 17 ulcers healed in median 24 mo [IQR:9–78]. In the remaining 13 (54%) pts 17 ulcers stayed as unhealed for a median 7 years [IQR:5–11], despite all interventions and medical treatment. We saw the mean age at BS onset was significantly younger (24.4±5.4 vs 30.4±7.3 years; p&lt;0.05) and the median time between disease onset and ulcer development was significantly shorter (3 [IQR:1–4.5] vs 10 [IQR:4–14 years, p&lt;0.05] in pts with healed ulcers compared to those with unhealed ulcers. There were no apparent associations with the type of management and the severity of venous involvement. <h3>Conclusions</h3> Leg ulcer develops mainly due to venous disease in the lower extremities. Peripheral arterial occlusive disease seems to be rarely involved. It may cause unemployment in 46% and be resistant to treatment in 54% of the cases. Ulcers that appear early during the disease course heal faster and is more responsive to treatment. <h3>Disclosure of Interest</h3> None declared