Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus
Chronic active infection with the Epstein-Barr virus (CAEBV) includes Hydro-like lymphoproliferative disorder (LPD-HV like) and severe allergic mosquitoes bite. LPD-HV like includes: classical vacciniform hydroa (HV), severe or systemic HV and hydroa-type lymphoma. Although of unknown etiology, gene...
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| Autores principales: | , , , , , , , |
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| Formato: | Artículo revista |
| Lenguaje: | Español |
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2019
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/25870 |
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| Sumario: | Chronic active infection with the Epstein-Barr virus (CAEBV) includes Hydro-like lymphoproliferative disorder (LPD-HV like) and severe allergic mosquitoes bite. LPD-HV like includes: classical vacciniform hydroa (HV), severe or systemic HV and hydroa-type lymphoma. Although of unknown etiology, genetic and environmental factors and EBV activation are postulated. It occurs in children and adolescents, predominantly in the male sex. It affects the photo-exposed skin of the face, back of the hands and earlobes, also mucous, and in advanced stages it can be generalized. Characterized by papule-vesicular rash, that ulcerate leaving a scar. Its highest incidence is in summer. They have a long clinical course with remission and recurrence, with risk of severe disease. The objective of this report is to announce a rare entity with variable prognosis.
Case presentation: 6-year-old male patient, with recurrent outbreaks of papules, vesicles and blisters that heal with atrophic scars, serohematic crusts in the malar area, ear pavilions, back of nose, back of hands and forearms, for 4 years, associated with ocular and oral involvement. Partial remissions and exacerbations to photo-exposure were referred, as well as fever and general decay on some occasions. A 0.7 x 0.5 cm skin losange biopsy was performed. Microscopy: blister with dermoepidermal level detachment with neutrophil polymorphonuclear cells and lymphocytes inside. The overlying epidermis shows intense reticular degeneration and keratinocyte necrosis. In the underlying dermis, moderate perivascular and diffuse inflammatory lymphocyte infiltrate is observed. Complementary methods: immunohistochemical positivity for EBER. Focal perforin and TIA1. Diagnosis: Vacciniform Hydroa. Given its evolution, it received treatment with corticosteroids, sun protection, and thalidomide, with good evolution. Currently without injuries and in follow-up.
The cutaneous processes associated with EBV, form a varied and sometimes overlapping pathological clinical spectrum. The correct interpretation gives the patient the opportunity of an adequate treatment, improving life quality and preventing more aggressive forms. |
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