RESUMO
We report the clinical and histopathological features of hydroa vacciniforme-like lymphoproliferative disorder in five indigenous and Mestizo children. All the children resided at higher altitudes, experiencing maximal solar exposure. All cases presented with prurigo along with Epstein-Barr virus infection. Histopathologic examination showed an atypical, CD30 + lymphocytic infiltrate with angiocentricity in all, while three cases demonstrated panniculitis-like infiltrate.
Assuntos
Infecções por Vírus Epstein-Barr , Hidroa Vaciniforme , Transtornos Linfoproliferativos , Humanos , Criança , Hidroa Vaciniforme/diagnóstico , Hidroa Vaciniforme/epidemiologia , Hidroa Vaciniforme/patologia , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/diagnóstico , Infecções por Vírus Epstein-Barr/epidemiologia , Herpesvirus Humano 4 , Equador/epidemiologia , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/epidemiologia , Transtornos Linfoproliferativos/patologiaRESUMO
BACKGROUND: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a chronic Epstein-Barr virus (EBV)-positive lymphoproliferative disease which may either present as an indolent condition or progress to a systemic T-cell lymphoma. METHODS: All HVLPD diagnosed over a 10-year period were retrieved, and clinical data regarding sex, age, oral and systemic manifestations, and clinical follow-up were obtained. Immunohistochemistry was done in order to characterize the lymphoid cells, and in situ hybridization was used to demonstrate the presence of EBV. RESULTS: Eleven cases were included, with a male predominance and a mean age of 25.1 years. Buccal mucosa and the lips were the most affected oral sites, appearing as painful ulcers. All patients exhibited facial oedema, usually affecting the lips, nose and periorbital region. The clinical course was gradual but progressive, with four patients having fever and 3 showing lymphadenopathies. All cases showed a moderate to severe lymphocytic infiltrate with angiotropism, angiocentricity and epidermotropism. Two cases affecting the lip skin exhibited a periappendageal lymphocytic infiltrate. Few large pleomorphic cells were found, surrounded by smaller and medium-sized lymphoid cells, as well as reactive plasma cells, macrophages, neutrophils and eosinophils. All lesions exhibited a cytotoxic T-cell (CD8+) phenotype with a variable proliferative index. All cases were associated with EBV, and all patients died due to complications of the disease. CONCLUSIONS: HVLPD is a rare disease that may show oral involvement with a cytotoxic T-cell phenotype, and is strongly associated with EBV. As shown in this series, HVLPD may show aggressive clinical behaviour.
Assuntos
Infecções por Vírus Epstein-Barr , Hidroa Vaciniforme , Transtornos Linfoproliferativos , Adulto , Infecções por Vírus Epstein-Barr/complicações , Feminino , Herpesvirus Humano 4 , Humanos , Masculino , PeruRESUMO
Hydroa vacciniforme (HV)-like lymphoma is a rare, usually fatal Epstein-Barr virus-driven lymphoproliferative disease affecting children from Asia, Mexico, and South America. Cutaneous manifestations imitate HV, a benign photodermatosis in which systemic symptoms are not observed, and spontaneous regression occurs later in adolescence or young adulthood. We report a case of HV-like lymphoma in a 12-year-old girl, descendent from an ancient Amazon indigenous tribe that, as far as we know, represents the second Brazilian case ever reported in the medical literature.
Assuntos
Hidroa Vaciniforme/patologia , Linfoma de Células T/patologia , Brasil , Criança , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/patologia , Feminino , Humanos , Hidroa Vaciniforme/virologia , Linfoma de Células T/virologiaRESUMO
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.
Assuntos
Infecções por Vírus Epstein-Barr/virologia , Herpesvirus Humano 4/isolamento & purificação , Hidroa Vaciniforme/virologia , Linfoma Cutâneo de Células T/virologia , Neoplasias Cutâneas/virologia , Adolescente , Adulto , Biomarcadores Tumorais/análise , Biópsia , Equador , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/patologia , Evolução Fatal , Feminino , Herpesvirus Humano 4/genética , Herpesvirus Humano 4/imunologia , Interações Hospedeiro-Patógeno , Humanos , Hidroa Vaciniforme/imunologia , Hidroa Vaciniforme/patologia , Imuno-Histoquímica , Hibridização In Situ , Linfoma Cutâneo de Células T/imunologia , Linfoma Cutâneo de Células T/patologia , Masculino , RNA Viral/genética , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/patologiaRESUMO
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.
Assuntos
Edema/patologia , Infecções por Vírus Epstein-Barr/complicações , Face/patologia , Neoplasias Faciais/patologia , Hidroa Vaciniforme/patologia , Linfoma Cutâneo de Células T/patologia , Adolescente , Adulto , Complexo CD3/análise , Criança , Pré-Escolar , Cicatriz/patologia , Cicatriz/virologia , Edema/virologia , Extremidades/patologia , Neoplasias Faciais/química , Neoplasias Faciais/virologia , Feminino , Humanos , Hidroa Vaciniforme/virologia , Imuno-Histoquímica , Linfoma Cutâneo de Células T/química , Linfoma Cutâneo de Células T/virologia , Masculino , México , Necrose/patologia , Necrose/virologia , Prognóstico , Tronco/patologia , Adulto JovemRESUMO
Hydroa vacciniforme-like lymphoma (HVL) is a rare cutaneous T-cell lymphoma that is usually seen in children of Hispanic or Asian origin. Association between chronic latent Epstein-Barr virus infection in both hydroa vacciniforme (HV) and HVL has been demonstrated and has recently been categorized by the World Health Organization as one of the Epstein Barr virus-positive lymphoproliferative disorders of childhood. Patients with HVL present with a cutaneous rash characterized by edema, blisters, ulcers, and scars mainly seen on the face and extremities that mimic HV; however, unlike in HV, the lesions tend to be extensive and deeper and are associated with severe scarring, necrosis, and systemic manifestations. We are reporting 7 cases of an unusual clinical variant of HVL with primarily periorbital edema. All of our patients in this series presented with progressive periorbital edema that was accompanied with systemic symptoms including fever, malaise, and lymphadenopathy. Most cases were initially misinterpreted as inflammatory processes including cellulitis, arthropod bite reactions, and periorbital lupus erythematosus. The biopsy of these lesions revealed an atypical lymphocytic infiltrate predominantly distributed in the deep dermis and in subcutaneous fat. Immunohistochemistry studies revealed a cytotoxic T-cell (CD8) profile. All cases were associated with Epstein-Barr virus infection. Our study presents a rare clinical variant of HVL with predominant periorbital edema. This variant could potentially be overlooked and misdiagnosed as an inflammatory condition; thus, it needs to be included in the differential diagnosis of periorbital edema in young patients.
Assuntos
Edema/patologia , Neoplasias Oculares/patologia , Hidroa Vaciniforme/patologia , Linfoma Cutâneo de Células T/patologia , Neoplasias Cutâneas/patologia , Adolescente , Biomarcadores Tumorais/análise , Biópsia , Criança , Diagnóstico Diferencial , Edema/etiologia , Edema/virologia , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/patologia , Infecções por Vírus Epstein-Barr/terapia , Infecções por Vírus Epstein-Barr/virologia , Neoplasias Oculares/química , Neoplasias Oculares/classificação , Neoplasias Oculares/terapia , Neoplasias Oculares/virologia , Feminino , Herpesvirus Humano 4/genética , Humanos , Hidroa Vaciniforme/classificação , Hidroa Vaciniforme/terapia , Hidroa Vaciniforme/virologia , Imuno-Histoquímica , Hibridização In Situ , Linfoma Cutâneo de Células T/química , Linfoma Cutâneo de Células T/classificação , Linfoma Cutâneo de Células T/terapia , Linfoma Cutâneo de Células T/virologia , Masculino , Valor Preditivo dos Testes , RNA Viral/genética , Neoplasias Cutâneas/química , Neoplasias Cutâneas/classificação , Neoplasias Cutâneas/terapia , Neoplasias Cutâneas/virologiaRESUMO
Se presenta caso de mujer de 14 años que consultó por úlceras orales y lesiones cutáneas de tipo vesicular, papular y algunas con evolución a úlcerocostras de centro necrótico, hemorrágico y de variadas formas y tamaños, distribuidas principalmente en áreas fotoexpuestas como rostro y extremidades superiores e inferiores. La anatomía patológica confirmó linfoma tipo hidroa vacciniforme atípico, considerado primer caso descrito y publicado en Paraguay.
A 14 year old female consulted with oral ulcers and skin lesions like vesicles and papules, some of them are ulcers and crusting with a necrotic and hemorrhagic center, the shape and size of every lesion is different, mainly they are presented in sun-exposed areas like the face, arms and legs. The histopathological finding confirms the atypical hidro vaccinifome-like lymphoma, considered first report and published in Paraguay.