Publication:

Aggressive mycosis fungoides/sézary syndrome in Nigeria: case report and literature review

Mycosis fungoides and its variant, sézary syndrome are rare neoplastic conditions which are part of a larger group of lymphomas that primarily affect the skin known as cutaneous T-cell lymphomas. We report a case of fatal aggressive mycosis fungoides/sézary syndrome in a 55-year old Nigerian man who initially developed pruritic hyperpigmented spots on his skin which progressed over the course of 5 years to widespread scaly mixed hyperpigmented and hypopigmented plaques and nodules with features of organ involvement despite being managed with Psoralen and ultraviolet A radiation (PUVA), total skin electron-beam (TSEB), local electron-beam radiation, bexarotene, and oral prednisolone and chlorambucil (Winklemann regimen). In the process, we highlight the rarity of the condition, its ease of misdiagnosis and its predilection for people of Sub-Saharan decent.