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

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 e...

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Bibliographic Details
Published in:BMJ Case Reports
Main Authors: Bedier, Helbies, Lin, John, Julien, Louis-André, Routy, Jean-Pierre
Format: Article in Journal/Newspaper
Language:English
Published: BMJ 2021
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Online Access:http://dx.doi.org/10.1136/bcr-2020-238644
https://syndication.highwire.org/content/doi/10.1136/bcr-2020-238644
Description
Summary: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.