Concurrent development of HIV-negative Kaposi’s sarcoma and mycosis fungoides in an elderly Inuit from Canada

• Published in: BMJ Case Reports
• Main Authors: Bedier, Helbies, Lin, John, Julien, Louis-André, Routy, Jean-Pierre
• Format: Text
• Language: English
• Published: BMJ Publishing Group 2021
• Subjects: Case Report; Canada; inuit
• Online Access: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7845731/; http://www.ncbi.nlm.nih.gov/pubmed/33509877; https://doi.org/10.1136/bcr-2020-238644

An 88-year-old Inuit man from Northern Canada presented with an extensive skin rash associated with numerous violaceous skin nodules on his palms and lower extremities. Biopsy of a skin nodule revealed Kaposi’s sarcoma (KS), a human herpesvirus 8 (HHV8)-associated malignancy, whereas biopsy of the erythematous skin showed an atypical infiltrate of CD4-positive T-cells that, together with TCR gene rearrangement and presence of clonal T-cells in peripheral blood by flow cytometry, was consistent with a T-cell lymphoma, mycosis fungoides (MF) subtype. Serology was negative for HIV and HTLV-I/II and no immunodeficiency syndrome was identified. The patient was successfully treated with an oral retinoid for KS, and with topical hydrocortisone and ultraviolet B (UVB) phototherapy for MF. This case highlights the existence of HHV8-related lesions in native persons of Northern Canada, and also that MF-induced immunosuppression combined with immunosenescence may play a role in the development of non-HIV-related KS.