皮膚病変，歯肉潰ようを認めた肺好酸球性肉芽腫様Ｌａｎｇｅｒｈａｎｓ ｃｅｌｌ ｇｒａｎｕｌｏｍａｔｏｓｉｓの１症例

Abstract

We report here a case of Langerhans cell granulomatosis resembling so-called pulmonary eosinophilic granuloma. Patient was a 38 year-old male with chief complaints of fever and general fatigue, and also showed skin lesions with crust and ulcer of gingiva. Chest X-ray film in hospitalization showed diffuse abnormal shadows which were not detected two months before the admission. In both lung fields, diffuse multiple small cystic lesions with thin wall, small nodules and patchy lesions were also seen on chest computed tomography. Abnormal shadows were dominant in upper lung fields. No other involvement of organs including bones were found by garium and bone scintigraphies.Biopsy specimens were obtained from transbronchial lung biopsy, skin and gingiva. Granuloma formation with Langerhans cells and eosinophils invasion were found in all biopsy specimens by hematoxylin-eosin staining and immunostaining against the S-100 protein. On the basis of these findings, we gave the preferable diagnosis of Langerhans cell granulomatosis rather than pulmonary eosinophilic granuloma, because the patient showed systemic involvement. However, main organ involved was the lung and the clinical findings were looked like pulmonary eosinophilic granuloma as a localized disease. Interestingly, without specific treatment excluding smoking cessation, clinical symptoms and chest X-ray abnormalities improved spontaneously in this case.