RESUMO
Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at higher risk of developing a severe course of infections especially viral and malignancies than the general population. The aim of the study was to present complex diagnostic and therapeutic management of HV-LPD. The clinical diagnosis was confirmed at the histological and molecular level with next generation sequencing. HV-LPD was diagnosed in a patient with CVID and chronic active Epstein-Barr virus (CAEBV) infection. The patient was refractory to CHOP chemotherapy and immunosuppressive treatment in combination with antiviral drugs (prednisone, bortezomib, gancyclovir). The third-party donor EBV-specific cytotoxic T cells (EBV-CTL, tabelecleucel) were used, which stabilised the disease course. Finally, matched unrelated donor hematopoietic cell transplantation (MUD-HCT) was performed followed by another cycle of EBV-CTL.
Assuntos
Imunodeficiência de Variável Comum , Infecções por Vírus Epstein-Barr , Hidroa Vaciniforme , Transtornos Linfoproliferativos , Neoplasias Cutâneas , Criança , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/terapia , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/terapia , Herpesvirus Humano 4 , Humanos , Hidroa Vaciniforme/diagnóstico , Hidroa Vaciniforme/terapia , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/terapiaRESUMO
Hydroa vacciniforme (HV) and solar urticaria (SU) are uncommon immunologically mediated photodermatoses. HV occurs almost exclusively in children, usually beginning in childhood and remitting spontaneously by adolescence. Association with chronic Epstein-Barr virus infection has been reported in HV, which raises the possibility of lymphoproliferative disorders in these patients. SU is characterized by skin erythema, swelling, and whealing immediately after sun exposure. Although several treatment options are available, the management of both conditions remains a challenge.
Assuntos
Hidroa Vaciniforme , Prevenção Primária/métodos , Protetores Solares/uso terapêutico , Raios Ultravioleta/efeitos adversos , Saúde Global , Humanos , Hidroa Vaciniforme/diagnóstico , Hidroa Vaciniforme/epidemiologia , Hidroa Vaciniforme/terapia , Morbidade/tendências , Roupa de ProteçãoRESUMO
Hydroa vacciniforme is a rare acquired photosensitivity disorder that manifests in childhood. We sought to identify the demographics, disease progression, symptoms, risk factors, and treatment efficacy associated with hydroa vacciniforme using a questionnaire. Nineteen surveys were included in the data analysis. Symptoms occurred throughout the year and the most common dermatologic manifestations included redness, blisters, bumps, scars, itching, and burning, predominantly on sun-exposed areas. The most common associated symptoms included oral ulcers, abdominal pain, and eye ulcers. Most participants limited their sun exposure to less than 10 hours weekly and stated sunscreen somewhat helped prevent breakouts. Most participants had no to limited improvement of their symptoms with treatments.
Assuntos
Hidroa Vaciniforme/patologia , Adolescente , Adulto , Idade de Início , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Hidroa Vaciniforme/diagnóstico , Hidroa Vaciniforme/epidemiologia , Hidroa Vaciniforme/prevenção & controle , Masculino , New York/epidemiologia , Fototerapia/métodos , Qualidade de Vida , Fatores de Risco , Protetores Solares/uso terapêutico , Inquéritos e Questionários , Resultado do TratamentoRESUMO
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