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Primary cutaneous aggressive epidermotropic CD8+ T‐cell lymphoma with KIR3DL2 and NKp46 expression in a human immunodeficiency virus carrier

Primary cutaneous aggressive epidermotropic T‐cell lymphoma (PCAETCL) is a very rare lymphoma characterized by rapidly growing necrotic cutaneous lesions with an epidermotropic CD8+ T‐cell neoplastic infiltrate observed histopathologically. It is associated with a very poor outcome, despite aggressive multi‐agent chemotherapy. We report a 49‐year‐old human immunodeficiency virus (HIV)‐infected patient who developed PCAETCL with associated marked vascular injury leading to diffuse purpuric and necrotic lesions complicated by recalcitrant hemophagocytic activation syndrome. The lymphoma strongly and diffusely expressed CD158k/KIR3DL2 at the protein and transcript level and NKp46 transcripts, in addition to CD8 and cytotoxic proteins. We observed a diffuse CD158k/KIR3DL2 protein expression in another case of PAETCL, not associated with immunodeficiency, which was used as a positive control. PCAETCL can develop in HIV‐infected patients and may present in vasculitis‐like fashion. The possible role of immunosuppression and/or HIV in oncogenesis can be postulated, as patients infected with HIV may develop anti‐HIV cytotoxic CD8+ lymphoproliferations. The frequency of CD158k/KIR3DL2 and NKp46 expression in PCAECL remains to be studied in a series of cases, and may represent interesting targets for future treatments.