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 2021-01, Vol.14 (1), p.e238644
Main Authors: Bedier, Helbies, Lin, John, Julien, Louis-André, Routy, Jean-Pierre
Format: Article
Language:English
Subjects:
Acitretin - therapeutic use
Administration, Cutaneous
Aged, 80 and over
Anemia
Anti-Inflammatory Agents - therapeutic use
Biopsy
Cancer
Case Report
Case reports
Cytomegalovirus
Dehydrogenases
Disease
Epstein-Barr virus
Flow cytometry
Fungal infections
Herpesvirus 8, Human
HIV
Human immunodeficiency virus
Humans
Hydrocortisone - therapeutic use
Immunocompromised Host
Immunosenescence
Inuit
Inuits
Kaposis sarcoma
Laboratories
Lymphocytes
Lymphoma
Male
Mycosis Fungoides - immunology
Mycosis Fungoides - pathology
Mycosis Fungoides - therapy
Neoplasms, Multiple Primary - immunology
Neoplasms, Multiple Primary - pathology
Neoplasms, Multiple Primary - therapy
Neutrophils
Patients
Population
Sarcoma
Sarcoma, Kaposi - drug therapy
Sarcoma, Kaposi - ethnology
Sarcoma, Kaposi - immunology
Sarcoma, Kaposi - pathology
Skin
Skin Neoplasms - immunology
Skin Neoplasms - pathology
Skin Neoplasms - therapy
Ultraviolet Therapy - methods
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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.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2020-238644