Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-cell Lymphoma in a Human T-cell Leukaemia Virus Type-1 Carrier
Primary cutaneous CD8 + aggressive epidermotropic cytotoxic T-cell lymphoma is rare, accounting for less than 1% of all cutaneous T-cell lymphoma (CTCL) (1). We report here a case of this type of lymphoma in a human T-cell leukaemia virus type-1 (HTLV-1) carrier. CASe rePorT A 58-year-old Japanese woman visited our hospital in 2006. She first noticed a red plaque on her left thigh approximately ten years previously. The number and the size of the plaques gradually increased despite treatment
... espite treatment with topical steroid. At the initial visit, dermatological examination revealed irregularly shaped elevated erythematous plaques and ulcers with pus and crusts on her right breast (Fig. 1a) . She also had multiple infiltrated red plaques with scales and crusts on her trunk and thigh (Fig. 1b) . No superficial lymph nodes were swollen. Her general condition was good. She was from Kyushu, Japan, an area endemic for HTLV-1, and she was seropositive for the virus. The peripheral white cell count was normal without any atypical lymphocytes. Laboratory values were within normal limits except for a slightly increased level of serum soluble IL-2 receptor (965 U/ml, normal: 167-497 U/ml). Systemic examination revealed no visceral involvement. A skin biopsy specimen from the ulcer on the right chest showed diffuse infiltration of leukocytes in the dermis and subcutaneous tissues. In the upper and middle part of the dermis, there was dense infiltrate of neutrophils, histiocytes and lymphocytes. Approximately half of infiltrating cells were CD3 + . These T cells were mainly CD4 + CD25 + , and the rest were CD8 + . Many CD8 + cells and some CD4 + CD25 + cells infiltrated the lower dermis and adipose tissues. By Southern blotting analyses, neither rearranged bands for T-cell receptor beta chain nor clonal integration of HTLV-1 were detected using DNA from the skin lesion. Considering her skin manifestation characteristic for CTCL, seropositivity of HTLV-1, and infiltration of CD4 + CD25 + cells in the skin, she was first diagnosed with smouldering type of adult T-cell leukaemia (ATL). Topical corticosteroid, psoralen plus ultraviolet A (PUVA) and oral retinoid were applied. These were partially effective, and the skin lesions disappeared 3 months after initiation of oral retinoid therapy except for the ulcers on the patient's right breast (Fig. 1c) and left thigh. She was subsequently treated with metho trexate instead of retinoid, but the Fig. 1. (a) Multiple elevated erythematous plaques and ulcers with pus and crusts on the right chest. (b) A red plaque on the left thigh. (c) A deep ulcer in the right breast that appeared 8 months after the patient's first visit. (d) Multiple purplered macules with central necrosis that appeared 14 months after her initial visit.