Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan.
Department of Pediatric Surgery, Graduate School of Medicine, Chiba University, Chiba, Japan.
Mod Rheumatol Case Rep. 2022 Jun 24;6(2):266-269. doi: 10.1093/mrcr/rxac020.
Behçet's disease (BD) is often associated with neutrophilic dermatosis. However, BD is rarely associated with aseptic abscesses in the spleen or liver. A 2-year-old girl presented to our hospital with a 2-week history of fever, abdominal pain, and a skin ulcer on her leg. Each time her skin was punctured with a needle for a blood test or spinal fluid test, she developed intractable aseptic abscesses on her skin. She was diagnosed with intestinal BD based on gastrointestinal endoscopy findings and was treated with prednisolone, mesalazine, and elemental diet therapy. Although these were effective for her colon ulcers, low-grade fever and mild abdominal pain persisted. Abdominal computed tomography revealed a low-density area in the spleen. Although it is recommended to check the contents with puncture drainage, it was difficult due to the risk of bleeding and pathergy. The abscess expanded despite antimicrobial therapy. We discontinued antimicrobial therapy and switched to intensified immunosuppressive therapy for BD [intravenous infliximab (IFX)]. After administration of IFX, the splenic abscess gradually disappeared, and all her symptoms improved. In cases of BD with splenic abscesses resistant to antimicrobial therapy, intensifying immunosuppressive therapy can be expected to shrink the abscesses and avoid splenectomy.
贝赫切特病（BD）常伴有中性粒细胞皮肤病。然而，BD 很少与脾或肝的无菌性脓肿有关。一名 2 岁女孩因发热、腹痛和腿部皮肤溃疡到我院就诊，病史为 2 周。每次她的皮肤被针刺进行血液检查或腰椎穿刺时，她的皮肤都会出现难治性无菌脓肿。根据胃肠镜检查结果，她被诊断为肠 BD，并接受了泼尼松龙、美沙拉嗪和要素饮食治疗。虽然这些治疗对她的结肠溃疡有效，但低热和轻度腹痛仍持续存在。腹部计算机断层扫描显示脾脏有低密度区域。虽然建议进行穿刺引流以检查内容物，但由于出血和穿刺易感性的风险，这很困难。尽管进行了抗菌治疗，脓肿仍在扩大。我们停止了抗菌治疗，转而对 BD（静脉注射英夫利昔单抗（IFX））进行强化免疫抑制治疗。IFX 给药后，脾脓肿逐渐消失，所有症状均改善。对于对抗菌治疗耐药的 BD 伴脾脓肿患者，强化免疫抑制治疗有望使脓肿缩小并避免脾切除术。
