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...
| 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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| Materias: | |
| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/25870 |
| Aporte de: |
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I10-R327-article-25870 |
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| record_format |
ojs |
| institution |
Universidad Nacional de Córdoba |
| institution_str |
I-10 |
| repository_str |
R-327 |
| container_title_str |
Revista de la Facultad de Ciencias Médicas de Córdoba |
| language |
Español |
| format |
Artículo revista |
| topic |
hidroa vacciniform virus Epstein Barr skin hidroa vacciniforme virus Epstein Barr piel |
| spellingShingle |
hidroa vacciniform virus Epstein Barr skin hidroa vacciniforme virus Epstein Barr piel Moyano Crespo, G Rodríguez, N Volmaro, K Suárez Ghibaudo, H Martínez , M Pets, E Meza Vetanzo, Z Herrero, MV Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus |
| topic_facet |
hidroa vacciniform virus Epstein Barr skin hidroa vacciniforme virus Epstein Barr piel |
| author |
Moyano Crespo, G Rodríguez, N Volmaro, K Suárez Ghibaudo, H Martínez , M Pets, E Meza Vetanzo, Z Herrero, MV |
| author_facet |
Moyano Crespo, G Rodríguez, N Volmaro, K Suárez Ghibaudo, H Martínez , M Pets, E Meza Vetanzo, Z Herrero, MV |
| author_sort |
Moyano Crespo, G |
| title |
Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus |
| title_short |
Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus |
| title_full |
Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus |
| title_fullStr |
Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus |
| title_full_unstemmed |
Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus |
| title_sort |
hydroa vacciniform as a cutaneous manifestation of the chronic active infection of epstein-barr virus |
| description |
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. |
| publisher |
Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2019 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/25870 |
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| first_indexed |
2024-09-03T21:01:13Z |
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2024-09-03T21:01:13Z |
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I10-R327-article-258702024-08-27T18:26:30Z Hydroa vacciniform as a cutaneous manifestation of the chronic active infection of Epstein-Barr virus Hidroa vaccinoforme como manifestación cutánea de la infección crónica activa del virus Epstein-Barr Moyano Crespo, G Rodríguez, N Volmaro, K Suárez Ghibaudo, H Martínez , M Pets, E Meza Vetanzo, Z Herrero, MV hidroa vacciniform virus Epstein Barr skin hidroa vacciniforme virus Epstein Barr piel 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. La infección crónica activa del virus Epstein-Barr (CAEBV) comprende el Desorden linfoproliferativo hidroa-like (LPD-HV like) y la picadura alérgica severa del mosquito. El LPD-HV like incluye: hidroa vacciniforme (HV) clásica, HV severa o sistémica y linfoma tipo hidroa. Aunque de etiología desconocida, se postulan factores genéticos, ambientales y la activación del EBV. Se presenta en niños y adolescentes, predominando en el sexo masculino. Afecta la piel foto-expuesta de la cara, el dorso de las manos y los lóbulos de las orejas, también mucosas, y en estadios avanzados puede ser generalizado. Caracterizada por erupción pápulo-vesiculosa que se ulceran dejando cicatriz. Su mayor incidencia es en verano. Tienen un curso clínico largo con remisión y recurrencia, con riesgo a enfermedad severa. El objetivo de este reporte es dar a conocer una entidad poco frecuente de pronóstico variable. Presentación del caso: paciente de sexo masculino de 6 años, presenta brotes recurrentes de pápulas, vesículas y ampollas que curan con cicatrices atróficas, costras serohemáticas en zona malar, pabellones auriculares, dorso de nariz, dorso de manos y antebrazos, desde hace 4 años, asociadas a compromiso ocular, y oral. Presentó remisiones parciales y exacerbaciones a foto-exposición. Refirió fiebre y decaimiento general en algunas oportunidades. Se realizó biopsia de losange de piel de 0,7 x 0,5 cm. Microscopía: piel con ampolla con despegamiento a nivel dermoepidérmico con neutrófilos polimorfonucleares y linfocitos en su interior. La epidermis suprayacente muestra intensa degeneración reticular y necrosis de los queratinocitos. En dermis subyacente se observa moderado infiltrado inflamatorio linfocitario perivascular y difuso. Métodos complementarios: registró positividad por inmunohistoquímica para EBER. Perforina y TIA1, focal. Diagnóstico: hidroa vacciniforme. Dada su evolución recibió tratamiento con corticoides, protección solar, y talidomida, con buena evolución. Actualmente sin lesiones y en seguimiento. Los procesos cutáneos asociados a EBV conforman un espectro clínico patológico variado y a veces superpuesto. La correcta interpretación brinda al paciente la oportunidad de un tratamiento adecuado mejorando la calidad de vida y previniendo formas más agresivas. Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2019-10-22 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/25870 Revista de la Facultad de Ciencias Médicas de Córdoba.; 2019: Suplemento JIC XX Revista de la Facultad de Ciencias Médicas de Córdoba; 2019: Suplemento JIC XX Revista da Faculdade de Ciências Médicas de Córdoba; 2019: Suplemento JIC XX 1853-0605 0014-6722 10.31053/1853.0605.v76.nSuplemento spa https://revistas.unc.edu.ar/index.php/med/article/view/25870/27698 Derechos de autor 2019 Universidad Nacional de Córdoba https://creativecommons.org/licenses/by-nc/4.0 |