title: Therapeutic efficacy of intravenous cyclophosphamide concomitant with moderate- to high-dose prednisolone in two patients with fasciitis panniculitis syndrome.
abstract: 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.
authors.affiliation: Institute of Rheumatology, Aoyama Hospital, Tokyo Women's Medical University, Tokyo, Japan. kintarou@s9.dion.ne.jp
authors.forename: Takashi
authors.initials: T
authors.lastname: Kato
authors.forename: Ayako
authors.initials: A
authors.lastname: Nakajima
authors.forename: Makoto
authors.initials: M
authors.lastname: Soejima
authors.forename: Reon
authors.initials: R
authors.lastname: Nagai
authors.forename: Toru
authors.initials: T
authors.lastname: Yago
authors.forename: Kiyoko
authors.initials: K
authors.lastname: Tanohara
authors.forename: Hisae
authors.initials: H
authors.lastname: Ichida
authors.forename: Ikuko
authors.initials: I
authors.lastname: Masuda
authors.forename: Toru
authors.initials: T
authors.lastname: Yamada
authors.forename: Atsuo
authors.initials: A
authors.lastname: Taniguchi
authors.forename: Yuji
authors.initials: Y
authors.lastname: Akiyama
authors.forename: Toshihide
authors.initials: T
authors.lastname: Mimura
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Tsuchida
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Kamatani
authors.forename: Masako
authors.initials: M
authors.lastname: Hara
mesh_terms: D000273:Adipose Tissue / Q000473:pathology; D000368:Aged; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004351:Drug Resistance / Q000187:drug effects; D004359:Drug Therapy, Combination; D005208:Fasciitis / Q000188:drug therapy* / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007275:Injections, Intravenous; D008297:Male; D008875:Middle Aged; D015434:Panniculitis / Q000188:drug therapy* / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use*; D012131:Respiratory Insufficiency / Q000188:drug therapy / Q000503:physiopathology; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone