Abstract
Hydroa vacciniforme-like cutaneous lymphoma is a very rare Epstein-Barr virus positive peripheral T-cell lymphoma affecting Asian and Hispanic children and young adults with a defective cytotoxic immune response to EBV predisposing to the development of the disease. We report on 2 Ecuadorian patients with papulovesicular and ulcerated crusted lesions on the face, upper and lower extremities and abdomen, with aggressive clinical course and, in one case, a fatal outcome. The histological and molecular profiles (immunohistochemistry and in situ hybridization) established a diagnosis of hydroa vacciniforme-like Epstein-Barr virus-encoded small RNAs + cutaneous T-cell lymphoma in both cases.
MeSH terms
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Adolescent
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Adult
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Biomarkers, Tumor / analysis
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Biopsy
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Ecuador
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Epstein-Barr Virus Infections / immunology
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Epstein-Barr Virus Infections / pathology
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Epstein-Barr Virus Infections / virology*
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Fatal Outcome
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Female
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Herpesvirus 4, Human / genetics
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Herpesvirus 4, Human / immunology
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Herpesvirus 4, Human / isolation & purification*
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Host-Pathogen Interactions
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Humans
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Hydroa Vacciniforme / immunology
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Hydroa Vacciniforme / pathology
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Hydroa Vacciniforme / virology*
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Immunohistochemistry
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In Situ Hybridization
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Lymphoma, T-Cell, Cutaneous / immunology
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Lymphoma, T-Cell, Cutaneous / pathology
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Lymphoma, T-Cell, Cutaneous / virology*
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Male
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RNA, Viral / genetics
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Skin Neoplasms / immunology
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Skin Neoplasms / pathology
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Skin Neoplasms / virology*
Substances
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Biomarkers, Tumor
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RNA, Viral