Abstract:

A 19-year-old Chinese woman presented with progressive ulcers and recurrent fever for 1 year. The lesions originally began with purplish red maculopapules, then rapidly developed into ulcer with severe pain and spread to the trunk and extremities accompanied by intermittent fever. Histopathology revealed lymphoid infiltration of middle and large atypical T cells throughout the dermis and subcutaneous fat layer along with focal epidermotropism. The lymphoid infiltrates were positive for CD3, CD8, T-cell intercellular antigen （TIA） and T-cell receptor β（TCR-β）. Gene analysis showed the rearrangement of T-cell receptor gene. According to the above findings, the patient was diagnosed as primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma. Combined therapy with cyclophosphamide, vincristine, prednisone and bleomycin resulted in partial remission of skin lesions, but she eventually died 22 months after the onset of disease.