Primary Cutaneous CD8-positive Aggressive Epidermotropic Cytotoxic T-cell Lymphoma Complicated by Paraneoplastic...

Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (PCAE-CTL) is a rare subtype of cutaneous T-cell lymphoma with a poor prognosis. We herein report a case of PCAE-CTL accompanied by anti-Ma2 antibody-positive paraneoplastic encephalitis. A 33-year-old woman with erythema and disturbance of consciousness was diagnosed with PCAE-CTL by a skin biopsy. Head magnetic resonance imaging revealed bilateral medial temporal lobe hyperintensity, and anti-Ma2 antibody in the cerebrospinal fluid was positive. She was diagnosed with concurrent anti-Ma2 antibody-positive paraneoplastic encephalitis. In cases of lymphoma with brain lesions, invasion of the central nervous system by lymphoma and paraneoplastic encephalitis should be considered.