Characterization of skin blister fluids from children with Epstein-Barr virus-associated lymphoproliferative disease

J Dermatol. 2018 Apr;45(4):444-449. doi: 10.1111/1346-8138.14226. Epub 2018 Jan 20.

Abstract

Epstein-Barr virus (EBV)-associated T- or natural killer (NK)-cell lymphoproliferative disease (LPD) is a heterogeneous group of disorders characterized by chronic proliferation of EBV-infected lymphocytes. Patients may present with severe skin manifestations, including hypersensitivity to mosquito bites (HMB) and hydroa vacciniforme (HV)-like eruption, which are characterized by blister formation and necrotic ulceration. Skin biopsy specimens show inflammatory reactions comprising EBV-infected lymphocytes. However, blister fluids have not been fully assessed in patients with this disease. Blister fluids were collected from three patients with EBV-associated LPD: two with HMB and one with HV. Immunophenotyping of blister lymphocytes and measurement of tumor necrosis factor (TNF)-α in blister fluids were performed. The patients with HMB and HV exhibited markedly increased percentages of NK and γδ T cells, respectively, in both peripheral blood and blister fluids. These NK and γδ T cells strongly expressed the activation marker human leukocyte antigen-DR and were considered to be cellular targets of EBV infections. TNF-α was highly elevated in all blister fluids. Severe local skin reactions of EBV-associated LPD may be associated with infiltrating EBV-infected lymphocytes and a high TNF-α concentration in blister fluids.

Keywords: Epstein-Barr virus; blister fluids; hydroa vacciniforme; hypersensitivity to mosquito bites; lymphoproliferative diseases.

Publication types

  • Case Reports

MeSH terms

  • Animals
  • Biopsy
  • Blister / immunology
  • Blister / pathology*
  • Blister / virology
  • Body Fluids / cytology*
  • Body Fluids / metabolism
  • Child
  • Epstein-Barr Virus Infections / immunology
  • Epstein-Barr Virus Infections / pathology*
  • Epstein-Barr Virus Infections / virology
  • HLA-DR Antigens / analysis
  • Herpesvirus 4, Human / isolation & purification*
  • Humans
  • Immunophenotyping
  • Intraepithelial Lymphocytes / immunology
  • Intraepithelial Lymphocytes / pathology
  • Killer Cells, Natural / immunology
  • Killer Cells, Natural / pathology
  • Lymphoproliferative Disorders / immunology
  • Lymphoproliferative Disorders / pathology*
  • Lymphoproliferative Disorders / virology
  • Male
  • Skin / cytology
  • Skin / pathology
  • Tumor Necrosis Factor-alpha / analysis

Substances

  • HLA-DR Antigens
  • Tumor Necrosis Factor-alpha