Sugihara Takahiko, Koike Rhuji, Nosaka Yurika, Ogawa Jun, Hagiyama Hiroyuki, Nagasaka Kenji, Nonomura Yoshinori, Nishio Junko, Nanki Toshihiro, Kohsaka Hitoshi, Kubota Tetsuo, Miyasaka Nobuyuki
Department of Bioregulatory Medicine and Rheumatology, Tokyo Medical and Dental University.
Nihon Rinsho Meneki Gakkai Kaishi. 2002 Jun;25(3):277-84. doi: 10.2177/jsci.25.277.
We report a case of a 27-year-old Japanese female with Sjogren's syndrome (SS), who suffered from several episodes of subcutaneous and mesenteric panniculitis with a recurrence within one year. After a history of fever and skin rash, the patient underwent surgery at a local hospital with a diagnosis of acute appendicitis complicated with an ileocecal abscess. She was also diagnosed as having SS. After the operation, the fever and skin rash persisted. She was treated with prednisolone (PSL), and her symptoms resolved. A recurrent bout of abdominal pain with fever, annular erythema on the trunk and a nodular erythematous rash on the lower extremities occurred six months after the operation. A skin biopsy from the lower extremities showed findings that were compatible with panniculitis. Abdominal computer tomography (CT) showed a diffuse swelling with soft tissue density in the intestinal mesenterium and para aortic area. A retrospective examination of the operative specimen obtained from the local hospital revealed centrilobular infiltration of neutrophils in the mesenteric adipose tissue with fat necrosis, which is compatible with mesenteric panniculitis. Twenty mg/day of PSL was successful in treating the systemic panniculitis, and the abnormal diffuse soft tissue density on the abdominal CT disappeared after three weeks of PSL administration. Systemic panniculitis is a rare complication in SS, and the pathogenesis is unclear.
我们报告一例27岁的日本女性干燥综合征（SS）患者，该患者在一年内反复出现几次皮下和肠系膜脂膜炎。在有发热和皮疹病史后，患者在当地医院接受手术，诊断为急性阑尾炎合并回盲部脓肿。她也被诊断为患有SS。术后，发热和皮疹持续存在。她接受了泼尼松龙（PSL）治疗，症状得到缓解。术后六个月，患者再次出现腹痛伴发热、躯干环形红斑和下肢结节性红斑皮疹。下肢皮肤活检显示的结果与脂膜炎相符。腹部计算机断层扫描（CT）显示肠系膜和腹主动脉旁区域有软组织密度的弥漫性肿胀。对从当地医院获取的手术标本进行回顾性检查发现，肠系膜脂肪组织中有中性粒细胞小叶中心浸润并伴有脂肪坏死，这与肠系膜脂膜炎相符。每天20毫克的PSL成功治疗了全身性脂膜炎，在给予PSL三周后，腹部CT上异常的弥漫性软组织密度消失。全身性脂膜炎是SS中一种罕见的并发症，其发病机制尚不清楚。
