title: Hydroa vacciniforme-like cutaneous T-cell lymphoma: clinicopathologic and immunohistochemical study of 12 cases.
abstract: BACKGROUND
  Hydroa vacciniforme (HV)-like lymphoma (HVL) is a rare and aggressive cutaneous T-cell lymphoma occurring mainly in children in Latin America and Asia. Chronic latent Epstein-Barr virus infection has been associated with both HV and HVL.
  
  
  OBJECTIVE
  We sought to evaluate the clinical presentation and histopathology of this rare cutaneous T-cell lymphoma.
  
  
  METHODS
  We reviewed the clinical, morphologic, and immunophenotypical features in 12 cases of HVL from Bolivia.
  
  
  RESULTS
  All 12 patients had skin lesions in both sun-exposed and nonsun-exposed areas, including edema, blistering, ulceration, and scarring, with a slowly progressive relapsing course. All 12 patients presented with systemic symptoms and showed a characteristic swelling of the nose and lips, and periorbital edema. Eight patients died an average of 5.3 months after initial diagnosis. Four patients remained alive with persistent disease. Histopathologic examination showed an atypical lymphocytic infiltrate with angiotropism and angiocentricity. The immunophenotype showed a cytotoxic T-cell (CD8(+)) profile. All cases were associated with Epstein-Barr virus infection and differed clinically from other forms of cutaneous T-cell lymphoma.
  
  
  CONCLUSIONS
  Only a limited number of cases were studied.
  
  
  CONCLUSIONS
  This study confirms that HVL is a highly aggressive lymphoma, although some patients have a more indolent, chronic course.
authors.affiliation: Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
authors.forename: Martin
authors.initials: M
authors.lastname: Sangueza
authors.forename: Jose Antonio
authors.initials: JA
authors.lastname: Plaza
mesh_terms: D000293:Adolescent; D000328:Adult; D018414:CD8-Positive T-Lymphocytes / Q000378:metabolism; D002648:Child; D003601:Cytotoxicity Tests, Immunologic; D005260:Female; D006801:Humans; D006837:Hydroa Vacciniforme / Q000276:immunology* / Q000473:pathology*; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017403:In Situ Hybridization; D016410:Lymphoma, T-Cell, Cutaneous / Q000276:immunology* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult