Ventilatory failure due to the limitation of chest movement in a case of FPS.

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  • Uehara Ritsuko
    Department of Rheumatic and Allergic Diseases, St, Marianna University School of Medicine
  • Suzuki Yasuo
    Department of Rheumatic and Allergic Diseases, St, Marianna University School of Medicine
  • Asanuma Yu
    Department of Rheumatic and Allergic Diseases, St, Marianna University School of Medicine
  • Ichikawa Yoichi
    Department of Rheumatic and Allergic Diseases, St, Marianna University School of Medicine
  • Abe Mitufumi
    Department of Pathology, St, Marianna University School of Medicine

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  • 皮下組織から筋組織にかけての線維化による胸郭運動制限のため，換気不全を来した筋膜炎‐脂肪織炎症候群（Ｆａｓｃｉｉｔｉｓ　Ｐａｎｎｉｃｕｌｉｔｉｓ　Ｓｙｎｄｒｏｍｅ）（ＦＰＳ）の一症例

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A 72-year-old woman presented with cervicothoracal skin lesions mimicked to scleroderma and muscular atrophy in 1996. Because of the elevation of serum creatinine kinase (CK), muscular biopsy was performed at another institution. Under the diagnosis of polymyositis, she was treated with corticosteroid. Despite of the decrease in serum CK levels by corticosteroid therapy, skin lesions and mascular dystrophy gradually worsened to extend to the regions of major pectoral, paravertebral, and femoral muscles. In 1997, she was admitted to our hospital because of dyspnea. On admission, the limitation of the chest movement was obvious and she developed respiratory arrest due to CO2 narcosis. The femoral magnetic resonace image (MRI) showed increased signal intensity of subcutaneous tissues and fascia on T 2-weighted image. The block biopsy specimens obtained from the cervical lesion revealed fibrotic thickness and chronic inflammation of subcutaneous septa, fascia, and perimysium. She was treated by mechanical ventilation and cimetidine and weekly methotrexate were added to the corticosteroid therapy because of the diagnosis of FPS. Thereafter, the skin and muscular lesions as well as the MRI findings were improved.<br> The concept of FPS was proposed by Naschitz et al. This condition is pathologically characterized by cicatrizing fascitis, septal and lobular panniculitis, and perimysial fibrosis and peripheral blood and tissue eosinophilia is not important for diagnosis. FPS includes classical eosinophilic fascitis but is also associated with several disorders such as malignancy. This case is suggestive of the therapeutic consideration of FPS in terms of the response to cimetidine and MTX.

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