title: Clinicopathologic Features of Hydroa Vacciniforme-Like Lymphoma: A Series of 9 Patients.
abstract: Hydroa vacciniforme-like lymphoma is a recently recognized cutaneous T-cell lymphoma associated with Epstein-Barr virus. The disease is observed in children of Latin American or Asian ethnicity. The authors report the clinical, histopathological, and immunophenotypical features of 9 new Mexican patients (M:F = 2:1; mean age, 14.5 years; median age, 13.3 years; age range, 4-27 years), expanding on previous observations of this elusive disease. The most common clinical aspects were persistent facial edema with necroses and pitted scars. Histopathological analyses revealed variably dense lymphoid infiltrates with common angiodestructive features. Neoplastic cells expressed CD3 and cytotoxic markers in all cases and were constantly positive for Epstein-Barr virus (EBER-1). Expression of other markers was variable. Follow-up data revealed that all patients died within 6 months or less, thus showing a very aggressive course with poor prognosis.
authors.affiliation: *Centre for Dermatology & Dermatopathology and School of Medicine, Universidad Nacional Autónoma de México/Hospital General de México "Eduardo Liceaga," México, DF, Mexico; and †Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.
authors.forename: Mario
authors.initials: M
authors.lastname: Magaña
authors.forename: Cesare
authors.initials: C
authors.lastname: Massone
authors.forename: Pablo
authors.initials: P
authors.lastname: Magaña
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cerroni
mesh_terms: D000293:Adolescent; D000328:Adult; D017252:CD3 Complex / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D002921:Cicatrix / Q000473:pathology / Q000821:virology; D004487:Edema / Q000473:pathology* / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005121:Extremities / Q000473:pathology; D005145:Face / Q000473:pathology*; D005153:Facial Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000821:virology; D005260:Female; D006801:Humans; D006837:Hydroa Vacciniforme / Q000473:pathology* / Q000821:virology; D007150:Immunohistochemistry; D016410:Lymphoma, T-Cell, Cutaneous / Q000737:chemistry / Q000473:pathology* / Q000821:virology; D008297:Male; D008800:Mexico; D009336:Necrosis / Q000473:pathology / Q000821:virology; D011379:Prognosis; D060726:Torso / Q000473:pathology; D055815:Young Adult
chemical_list: D017252:CD3 Complex