[Unusual course of CD8+ epidermotropic cutaneous T-cell lymphoma]

Aggressive epidermotropic cutaneous T-cell lymphoma (AECL) is a rare and aggressive form of lymphoma that exhibits systemic spread within a few months that is not preceded by any indolent form. Herein, we report a case of AECL occurring on foot lesions present for six years, and initially diagnosed as Woringer-Kolopp disease, or pagetoid cutaneous T-cell lymphoma.

A male patient presented an ulcerated lesion of the ankle that had been present for six years. Biopsy revealed pagetoid migration of CD8+, CD2-, CD5-, CD7+, CD30- and CD56- lymphocytes with expression of cytotoxic markers and of Ki67 in over 60% of cells. The resulting diagnosis was one of pagetoid cutaneous T-cell lymphoma, also known as Woringer-Kolopp disease. Despite treatment with methotrexate and carmustine, the ulcer worsened rapidly within two months. Subsequent biopsy revealed epidermal and dermal infiltration with large cells of identical phenotype to that seen in the previous biopsy, with angiocentrism and expression of Ki67 in over 90% of cells, pointing to a diagnosis of AECL. Progression to disseminated ulceronecrotic lesions occurred rapidly, and the patient died of sepsis within a few months.

AECL is characterised by ulcerative-haemorrhagic lesions that develop aggressively without any preceding mild cutaneous lesions. Median survival is 12 months. Histological analysis shows pagetoid epidermotropism comprising large monomorphic CD8+, CD2- and CD5- cells with markers for cytotoxicity and high expression of Ki67. The initial indolent phase in the case we report herein accounts for the diagnostic confusion at the outset with Woringer-Kolopp disease. Negative status of CD2 and CD5 labels may allow prompt diagnosis of AECL.