Kato Takashi, Nakajima Ayako, Soejima Makoto, Nagai Reon, Yago Toru, Tanohara Kiyoko, Ichida Hisae, Masuda Ikuko, Yamada Toru, Taniguchi Atsuo, Akiyama Yuji, Mimura Toshihide, Tsuchida Tetsuya, Kamatani Naoyuki, Hara Masako
Institute of Rheumatology, Aoyama Hospital, Tokyo Women's Medical University, Tokyo, Japan.
Mod Rheumatol. 2008;18(2):193-9. doi: 10.1007/s10165-008-0027-2. Epub 2008 Feb 20.
Fasciitis panniculitis syndrome (FPS) has been proposed as a new category of 'fasciitis' and includes the well-established eosinophilic fasciitis (EF). Unlike EF, FPS exhibits inconsistent eosinophilia and/or eosinophilic infiltration of the lesions. Principal histological FPS findings include dermal thickening, inflammation and thickening of the subcutaneous fat tissue, fibrous thickening of the fascia and inflammation of the adjacent muscle. FPS is commonly resistant to corticosteroids, and cimetidine is effective in approximately 80% of FPS patients. A new therapy for FPS is required for cases refractory to treatment or intolerant to cimetidine because of adverse drug reaction. In this report, two FPS patients were resistant to corticosteroids. Both received intravenous cyclophosphamide (IVCY) concomitant with moderate- to high-dose prednisolone (PSL), and this effectively treated the induration of the FPS lesions. Patient 1 was a 50-year-old woman who had been diagnosed with fasciitis following en bloc muscle biopsy of the thigh. She had been treated with high-dose PSL for 6 years, but the fasciitis was refractory. Induration of the neck, thorax and thighs resulted in impaired neck rotation, restrictive respiratory failure and impaired walking. A diagnosis of FPS was made by re-assessing the en bloc muscle biopsy. Although PSL (40 mg/day) for 18 days was ineffective, the addition of IVCY (400 mg) dramatically improved the disease manifestations. Patient 2 was a 68-year-old man who was diagnosed with fasciitis based on en bloc muscle biopsy of the left foot. He had been treated with PSL for 16 years, but the fasciitis was refractory. He exhibited lower limb induration and a refractory skin ulcer of the left foot. A diagnosis of FPS was made by re-assessing the en bloc muscle biopsy. Although PSL (40 mg/day) for 2 weeks was ineffective, the addition of IVCY (450 mg) improved both the lower limb induration and the skin ulcer. FPS may cause both entrapment vasculopathy of subcutis and perivasculitis of the subcutaneous fat tissue such that the skin ulcer might be closely related with the ischemic mechanism triggered by FPS. According to the clinical courses of our cases, IVCY combined with moderate- to high-dose PSL may be a new therapeutic choice for corticosteroid-resistant FPS patients.
脂膜炎性筋膜炎综合征（FPS）已被提议作为一种新的“筋膜炎”类别，其中包括已被充分认识的嗜酸性筋膜炎（EF）。与EF不同，FPS的嗜酸性粒细胞增多和/或病变处嗜酸性粒细胞浸润情况不一致。FPS主要的组织学表现包括真皮增厚、皮下脂肪组织炎症和增厚、筋膜纤维增厚以及相邻肌肉炎症。FPS通常对皮质类固醇耐药，而西咪替丁对约80%的FPS患者有效。对于治疗难治或因药物不良反应而不耐受西咪替丁的病例，需要一种新的FPS治疗方法。在本报告中，两名FPS患者对皮质类固醇耐药。两人均接受静脉注射环磷酰胺（IVCY）并联合中高剂量泼尼松龙（PSL）治疗，这有效治疗了FPS病变的硬结。患者1是一名50岁女性，在对大腿进行整块肌肉活检后被诊断为筋膜炎。她接受高剂量PSL治疗6年，但筋膜炎难治。颈部、胸部和大腿的硬结导致颈部旋转受限、限制性呼吸衰竭和行走障碍。通过重新评估整块肌肉活检做出了FPS的诊断。尽管给予18天的PSL（40毫克/天）无效，但加用IVCY（400毫克）后疾病表现显著改善。患者2是一名68岁男性，根据对左脚的整块肌肉活检被诊断为筋膜炎。他接受PSL治疗16年，但筋膜炎难治。他表现为下肢硬结和左脚难治性皮肤溃疡。通过重新评估整块肌肉活检做出了FPS的诊断。尽管给予2周的PSL（40毫克/天）无效，但加用IVCY（450毫克）改善了下肢硬结和皮肤溃疡。FPS可能导致皮下血管受压病变和皮下脂肪组织血管周围炎，使得皮肤溃疡可能与FPS引发的缺血机制密切相关。根据我们病例的临床病程，IVCY联合中高剂量PSL可能是对皮质类固醇耐药的FPS患者的一种新的治疗选择。
