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

BMJ Case Rep. 2021 Jan 28;14(1):e238644. doi: 10.1136/bcr-2020-238644.

Abstract

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.

Keywords: dermatology; haematology (incl blood transfusion); immunology; infectious diseases.

Publication types

  • Case Reports

MeSH terms

  • Acitretin / therapeutic use
  • Administration, Cutaneous
  • Aged, 80 and over
  • Anti-Inflammatory Agents / therapeutic use
  • Herpesvirus 8, Human
  • Humans
  • Hydrocortisone / therapeutic use
  • Immunocompromised Host
  • Immunosenescence
  • Inuit*
  • Male
  • Mycosis Fungoides / immunology
  • Mycosis Fungoides / pathology*
  • Mycosis Fungoides / therapy
  • Neoplasms, Multiple Primary / immunology
  • Neoplasms, Multiple Primary / pathology*
  • Neoplasms, Multiple Primary / therapy
  • Sarcoma, Kaposi / drug therapy
  • Sarcoma, Kaposi / ethnology
  • Sarcoma, Kaposi / immunology
  • Sarcoma, Kaposi / pathology*
  • Skin Neoplasms / immunology
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy
  • Ultraviolet Therapy / methods

Substances

  • Anti-Inflammatory Agents
  • Acitretin
  • Hydrocortisone