A 42-years old man was admitted to Peking Union Medical College Hospital because of blisters and mucosa erosions on both his lip and oral cavity for one month, he was diagnosed as “Behcet disease” and treated with cortisone. When cortisone was tapered down, his erosions exacerbated. Four months ago, a mass was found in his mediastinum, the mass was completely resected and the initial pathological report was lymphoproliferative disease with local malignant alteration. Physical examination showed multiple erosions on his lips, eyes, nose and genitals and scarce hair (Figure 1A). A biopsy of his scalp skin was made. The indirect immunofluorescence (IIF) by monkey esophageal epithelium revealed deposition of IgG both inter the keratinocyte cell and along the basement membrane zone with a titer of 1:1280 (Figure 1B). IIF of the patient’s serum with rat bladder as a substrate was also strong positive. The diagnosis of PNP was made. Methylprednisolone was applied intravenously 40 mg daily and intravenous immunoglobulin was also given. His mucosal lesions were improved. Somatostatin receptor scintillography revealed a high expression mass of 4.5 cm × 4 cm in diameter in his mediastinum. Pathologists retrialed the specimen of firstly resected tumor, biopsy sections of the tumor showed spindle to round cell with indistinct cell borders and central nuclei. The tumor cells tended to form fascicles and whorls in a storiform pattern (Figure 1C). Some areas of necrosis were present. On immunohistochemistry, the tumor cells of the sarcoma had an S-100++ (Figure 1D), Vimentin+, CD68+ (Figure 1E), CD35–, CD21–, CD1a-phenotype. The ki-67 index was 67%. The background small lymphocytes are predominantly CD3+ T-cells with only very few CD20+ B-cells. The final diagnosis was IDCS. Bone marrow aspirate, including flow cytometry, and biopsy revealed no evidence of leukemic involvement. A second surgery was suggested, but the patient refused. He received two cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). However, he developed severe pneumonia during the period of bone marrow suppression and died of respiratory failure about 7 months after surgery.
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