title: Hydroa vacciniforme and hydroa vacciniforme-like T-cell lymphoma: an uncommon event for transformation.
abstract: BACKGROUND
  Hydroa vacciniforme (HV) is associated with Epstein-Barr virus (EBV) infection and a risk of transformation to lymphoma.
  
  
  METHODS
  We retrospectively analyzed six HV cases for EBV association and transformation to HV-like T-cell lymphoma. Clinicopathologic features were reviewed and cases were assessed for EBV-encoded RNA (EBER) by in situ hybridization, double staining with immunohistochemistry and EBER and for T-cell clonality.
  
  
  RESULTS
  The male-to-female ratio was 5:1, with a median age at diagnosis of 18.5 years. All patients initially had recurrent vesicles, necrotic ulcers or scars on sun-exposed areas. Symptoms were present before diagnosis between 2 weeks to 10 years. The mean follow-up time was 106.3 months. Four patients (67%) were EBV-positive. All four EBV-positive and one EBV-negative patients had relapsing clinical course. Double staining proved EBV infection in T-cells. Moreover, one EBV-positive patient developed HV-like T-cell lymphoma with hemophagocytosis after 209 months of recurrent papulovesicular eruptions and eventually died. T-cell clonality was successfully performed in four HV patients and all showed polyclonal results; the transformed HV-like T-cell lymphoma was monoclonal.
  
  
  CONCLUSIONS
  In EBV endemic areas, HV is frequently (67%) associated with EBV infection, but transformation to HV-like T-cell lymphoma seems to be uncommon (17%) and bear a dismal outcome.
authors.affiliation: Department of Pathology, Chia-Yi Christian Hospital, Chia-Yi, Taiwan.|Department of Cosmetic Science, Chia Nan University of Pharmacy and Science, Tainan, Taiwan.
authors.forename: Chien-Chin
authors.initials: CC
authors.lastname: Chen
authors.affiliation: Department of Pathology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan.
authors.forename: Kung-Chao
authors.initials: KC
authors.lastname: Chang
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: L Jeffrey
authors.initials: LJ
authors.lastname: Medeiros
authors.affiliation: Department of Dermatology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan.
authors.forename: Julia Yu-Yun
authors.initials: JY
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D002472:Cell Transformation, Viral; D002648:Child; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D006837:Hydroa Vacciniforme / Q000473:pathology / Q000821:virology*; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D016399:Lymphoma, T-Cell / Q000821:virology*; D008297:Male; D016133:Polymerase Chain Reaction; D055815:Young Adult
keywords: Epstein-Barr virus; HV-like T-cell lymphoma; T-cell clonality; hydroa vacciniforme (HV); transformation