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Adult T-cell leukemia/lymphoma (ATLL) is a malignant lymphoproliferative disorder caused by human T-cell leukemia virus type 1 (HTLV-1).HTLV-1 is endemic in south-western Japan and the Caribbean basin.HTLV-1-induced ATLL has been divided in 4: hematological subtypes as acute, lymphoma, chronic, and smoldering.Chronic and smoldering ATLL have a relatively good prognosis, even without treatment.These types can, however, evolve to acute ATLL,which has a poor prognosis: the median survival time (MST) after ATLLdiagnosis is only 6 months.Specific skin lesions are caused by infiltration of the skin by the tumor cells and have been described in about half of patients with ATLL.We previously studied 124 cases of ATLL with specific skin lesions.We found that the smoldering ATLL with skin involvements may have a worse prognosis than that without skin lesions.In 1992, we reported that differences in HTLV-1 integration patterns between skin lesions and peripheral blood lymphocytes of ATLL patients with cutaneous involvements.Later, some reports have described patients who presented with only the cutaneous lesions without either leukemic change or visceral invasion.So we proposed a fifth category, cutaneous ATLL.Cutaneous ATLL show less than 4× 109/L lymphocyte in peripheral blood and nomonoclonal outgrowth ofT lymphocyte in peripheral blood,lymph node and organs other than skin at diagnosis.We collected 92 cases fit category of cutaneous ATLL, which showed that the MST after diagnosis is only 18.5 months.In conclusion, cutaneous ATLL could be a distinct entity that should be separated from the smoldering ATLL.It should be classified as another subtype in ATLL.