[症例報告]皮膚 Histiocytosis X の1症例
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A 64-Year-old woman came to the hospital for anal pain. Physical examination disclosed an ulcerative lesion at the anus. Laboratory findings on admission were within normal range except for ASLO (+), ALP 21.5U/1, and a erythrocyte sedimentation rate of 37mm/h. X-ray examination of bone and colon did not show any abnormality. Histological examination of the resected specimen showed a band-like, solid proliferation of histiocyte like cells along the ulcer base. These cells were large, rounded cells with abundant, slightly eosinophilic cytoplasm. The nucleus was oval or indented or kidney shaped. The nucleolus was not prominant. Both PAS and sudan III stains were negative. Electron microscopically Birbeck granules were seen in the cytoplasm of the cells. But tonofilaments or melanosomes were not found. In addition, junctional apparatus as desmosome was not observed. Immunohistochemical examination by PAP method of Sternberger revealed a positive for S1OO protein and a negative for lysozyme and $ \alpha $1 anti-trypsin. These results indicate that the histiocyte like cells are Langerhans cells. Langerhans cells have been considered to be a member of the mononuclear phagocytic system, because of the expression of similar immunological markers such as Fc and C3 receptors which are common in both Langerhans cells and cells in the mononoclear phagocytic system. But recent reports suggest some differences between Langerhans cells and cells in the monuclear phagocytic system. Langerhans cells are S1OO protein +, lysozyme -, NCA (nonspecific cross reacting antigen with carcinoembryonic antigen) -, whereas monocytes and macrophages are S1OO protein -, lysozyme +, NCA +. Watanabe. S., et al propose the hypothesis that there are two histiocytic cell lines; one is the monocyte-macrophage system, another is the T-zone histiocyte system. The cells in the T-zone histiocyte system include Langerhans cell and indeterminate cell in the epidermis and interdigitating cell in the lymph node and spleen. The function of the T-zone histiocyte is still unknown. However it is very interesting that the celle of the T-zone histiocyte increase in number, associated with the diseases related to the allergy or immuunological abnormality or with lympho-proliferative disorders as mycosis fungoides.
- 琉球大学医学部,Faculty of Medicine, University of the Ryukyusの論文
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- [症例報告]皮膚 Histiocytosis X の1症例