Abstract

Abstract We report a rare case of primary cutaneous CD8+ aggressive epidermotrophic cytotoxic T-cell lymphoma with striking clinical and histopathological features. A 51-year-old man, originally from Cameroon, presented to the department with a 6-week history of a progressive, itchy papulosquamous eruption that started on the lower abdomen shortly following infection with COVID-19. It became widespread over a short number of weeks, with blistering and ulceration in some areas. The patient reported fatigue with an intermittent low-grade pyrexia, but no systemic symptoms otherwise, and he was previously fit and well. Physical examination revealed an extensive, infiltrated papulonodular eruption with thick scale that was more marked in the skinfolds and involving the acral surfaces. Histology from three morphologically different sites showed marked epidermal hyperplasia and a prominent epidermotrophic infiltrate of medium-sized atypical lymphoid cells. There were frequent mitotic figures and evidence of apoptosis. Immunohistochemistry revealed that the atypical lymphoid cells in the epidermis were T cells expressing CD3, CD7 and CD8, together with cytotoxic markers and a high Ki67 proliferation fraction. These features were consistent with a diagnosis of CD8+ aggressive epidermotrophic cytotoxic T-cell lymphoma. Initial bloods and imaging were fairly unremarkable. Since the diagnosis, the patient has been treated with multiple types and courses of chemotherapy, with relapses in between. Most recently, the patient has been referred for total skin electron beam therapy with investigations for allogenic stem cell transplant ongoing. Owing to its rarity, primary cutaneous CD8+ aggressive epidermotrophic cytotoxic T-cell lymphoma is categorized only as a provisional entity in the most recent World Health Organization classification (Gilson D, Whittaker SJ, Child FJ et al. British Association of Dermatologists and U.K. Cutaneous Lymphoma Group Guidelines for the management of primary cutaneous lymphomas 2018. Br J Dermatol 2018; 180:496–526). Despite a variable and polymorphic presentation, the histology is distinctive in showing monomorphous, medium-sized atypical lymphocytes in the epidermis. We discuss the epidemiology of the disease and the paucity of therapeutic guidelines. In addition, our patient’s disease developed soon after COVID-19 infection; hence, we review the growing literature on the association between COVID-19 infection and vaccination and diagnosis, and relapse and remission of cutaneous T-cell lymphoma.

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