Hydroa vacciniforme-like lymphoproliferative disorder: Clinicopathologic study of 41 cases

J Am Acad Dermatol. 2019 Aug;81(2):534-540. doi: 10.1016/j.jaad.2019.01.011. Epub 2019 Jan 14.


Background: Hydroa vacciniforme-like lymphoproliferative disorder (HVLLPD) is a rare Epstein-Barr virus (EBV)-related disease that is usually found in East Asians and Latin Americans.

Objective: To report the characteristics of HVLLPD in Chinese patients.

Methods: Retrospective analysis of patients with HVLLPD from a single institute.

Results: A total of 41 patients were enrolled. All patients presented with papulovesicular lesions, mainly distributed on sun-exposed areas, with 26 patients showing systemic symptoms. Follow-up data were available for 20 patients, 16 patients were alive, and 4 patients died. Of the 4 deceased patients, 3 had taken a serum EBV DNA test that showed high viral loads. These 3 patients also received chemotherapy. Histopathology was characterized by dense proliferation of lymphocytes in the dermis. Angiotropism or angiodestruction was found in the majority of patients, whereas prominent cellular polymorphism was noticed in only 4 patients. All patients were positive for CD3, TIA1 cytotoxic granule associated RNA binding protein, and EBV-encoded RNA in situ hybridization.

Limitations: This was a retrospective study.

Conclusions: HVLLPD in Chinese patients showed indolent behavior in the majority of cases, which differed from the characteristics of HVLLPD in Latin Americans. Patients with high serum EBV DNA loads had an increased risk of their disease evolving into aggressive disease. Chemotherapy should not be considered as first-line treatment for most Chinese patients.

Keywords: Epstein-Barr virus; cutaneous lymphoma; hydroa vacciniforme; hydroa vacciniforme–like lymphoma; hydroa vacciniforme–like lymphoproliferative disorder.

MeSH terms

  • Adolescent
  • Adult
  • Antigens, CD / metabolism
  • CD4-CD8 Ratio
  • Child
  • Child, Preschool
  • China
  • DNA, Viral / blood*
  • Epstein-Barr Virus Infections / blood
  • Epstein-Barr Virus Infections / complications*
  • Female
  • Herpesvirus 4, Human*
  • Humans
  • Hydroa Vacciniforme / pathology
  • Infant
  • Lymphocytes / metabolism
  • Lymphoproliferative Disorders / blood*
  • Lymphoproliferative Disorders / complications
  • Lymphoproliferative Disorders / pathology*
  • Lymphoproliferative Disorders / virology
  • Male
  • Retrospective Studies
  • Viral Load
  • Young Adult


  • Antigens, CD
  • DNA, Viral