Clinicoprognostic Study of Hydroa Vacciniforme-like Lymphoproliferative Diseases: A Systematic Review

Dermatology. 2023;239(5):700-711. doi: 10.1159/000530607. Epub 2023 May 25.

Abstract

Background: Limited clinicopathological and prognostic data are available on hydroa vacciniforme (HV)-like lymphoproliferative diseases (HVLPD).

Methods: This systematic review searched HVLPD reports in Medline via PubMed, Embase, Cochrane, and CINAHL databases in October 2020.

Results: A total of 393 patients (65 classic HV, 328 severe HV/HV-like T-cell lymphoma [HVLL]) were analyzed. Among severe HV/HVLL cases, 56.0% were Asians, whereas 3.1% were Caucasians. Facial edema, hypersensitivity to mosquito bites, the onset of skin lesion, and percentage of severe HV/HVLL differed significantly by race. Progression to systemic lymphoma was confirmed in 9.4% of HVLPD patients. Death occurred in 39.7% patients with severe HV/HVLL. Facial edema was the only risk factor associated with progression and overall survival. Mortality risk was higher in Latin Americans than in Asians and Caucasians. CD4/CD8 double-negativity was significantly associated with the worst prognosis and increased mortality.

Conclusion: HVLPD is a heterogeneous entity with variable clinicopathological features associated with genetic predispositions.

Keywords: Hydroa vacciniforme-like lymphoproliferative diseases; Lymphoma; Photodermatoses; Prognosis; Systematic review.

Publication types

  • Systematic Review

MeSH terms

  • Edema
  • Epstein-Barr Virus Infections* / complications
  • Epstein-Barr Virus Infections* / pathology
  • Herpesvirus 4, Human / genetics
  • Humans
  • Hydroa Vacciniforme* / complications
  • Hydroa Vacciniforme* / diagnosis
  • Hydroa Vacciniforme* / pathology
  • Lymphoproliferative Disorders* / diagnosis
  • Lymphoproliferative Disorders* / pathology