Pênfigo neonatal em filho de mãe com pênfigo vulgar: relato de caso / Neonatal pemphigus in an infant born to a mother with pemphigus vulgaris: a case report

RESUMO Objetivo: Relatar um caso de pênfigo neonatal em paciente que manifestou lesões cutâneas extensas e críticas ao nascimento. Descrição do caso: Recém-nascido do sexo masculino com lesões vesicobolhosas extensas em região anterior do tórax e abdome, desde o nascimento. Admitido na ala pediátrica de um hospital para diagnóstico etiológico e tratamento. Com base na história materna e na avaliação clínica, concluiu tratar-se de pênfigo vulgar neonatal. O paciente apresentou evolução satisfatória, sem a necessidade de intervenção farmacológica. Comentários: Os casos descritos na literatura e as referências avaliadas revelam o pênfigo neonatal como uma doença de ocorrência rara, porém cujo conhecimento e diagnóstico precoce têm grande relevância clínica, considerando-se que geralmente se manifesta com lesões epidérmicas extensas e de aspecto crítico, embora apresente curso clínico transitório e benigno, sem necessidade de tratamento específico e sem relação com doença futura.

ABSTRACT Objective: To report on the case of a patient with neonatal pemphigus that had extensive and critical skin lesions at birth. Case description: A newborn male with extensive vesico-bullous lesions on the anterior side of his chest and abdomen at birth. He was admitted to the pediatric ward of a hospital for an etiological diagnosis and for treatment. Based on maternal history and a clinical evaluation, the patient was diagnosed with neonatal vulgar pemphigus. His progression was satisfactory and, in the end, he did not need pharmacological interventions. Comments: The cases reported in the literature and the references evaluated reveal that neonatal pemphigus is rare, but that knowledge about the disease allows for an early diagnosis to be made. This has great clinical relevance considering that the disease usually manifests itself in the form of extensive epidermal lesions, even though it is transient and benign, it does not require specific treatment, and it does not have any relation with possible future diseases.

NEONATAL PEMPHIGUS IN AN INFANT BORN TO A MOTHER WITH PEMPHIGUS VULGARIS: A CASE REPORT. / PÊNFIGO NEONATAL EM FILHO DE MÃE COM PÊNFIGO VULGAR: RELATO DE CASO.

OBJECTIVE: To report on the case of a patient with neonatal pemphigus that had extensive and critical skin lesions at birth. CASE DESCRIPTION: A newborn male with extensive vesico-bullous lesions on the anterior side of his chest and abdomen at birth. He was admitted to the pediatric ward of a hospital for an etiological diagnosis and for treatment. Based on maternal history and a clinical evaluation, the patient was diagnosed with neonatal vulgar pemphigus. His progression was satisfactory and, in the end, he did not need pharmacological interventions. COMMENTS: The cases reported in the literature and the references evaluated reveal that neonatal pemphigus is rare, but that knowledge about the disease allows for an early diagnosis to be made. This has great clinical relevance considering that the disease usually manifests itself in the form of extensive epidermal lesions, even though it is transient and benign, it does not require specific treatment, and it does not have any relation with possible future diseases.

OBJETIVO: Relatar um caso de pênfigo neonatal em paciente que manifestou lesões cutâneas extensas e críticas ao nascimento. DESCRIÇÃO DO CASO: Recém-nascido do sexo masculino com lesões vesicobolhosas extensas em região anterior do tórax e abdome, desde o nascimento. Admitido na ala pediátrica de um hospital para diagnóstico etiológico e tratamento. Com base na história materna e na avaliação clínica, concluiu tratar-se de pênfigo vulgar neonatal. O paciente apresentou evolução satisfatória, sem a necessidade de intervenção farmacológica. COMENTÁRIOS: Os casos descritos na literatura e as referências avaliadas revelam o pênfigo neonatal como uma doença de ocorrência rara, porém cujo conhecimento e diagnóstico precoce têm grande relevância clínica, considerando-se que geralmente se manifesta com lesões epidérmicas extensas e de aspecto crítico, embora apresente curso clínico transitório e benigno, sem necessidade de tratamento específico e sem relação com doença futura.

Osteosarcoma in patients younger than 12 years old without metastases have similar prognosis as adolescent and young adults.

BACKGROUND: Childhood cancer is relatively rare and tends to present specific age distribution, as a prognostic factor for some of these diseases. Information on how young age affects prognosis, response to chemotherapy, and local control options in children versus AYA with osteosarcoma (OST) is minimal. METHODS: In order to identify the main differences in clinical pathologic features, surgical approaches and survival rates of primary high grade OST of the extremity between children (n = 156; <12 years old) and AYA (n = 397; 12-30 years old), the institutional database with 553 patients treated by BOTG studies over 15 years were reviewed. RESULTS: There were no differences in metastases at diagnosis, tumor size, and grade of necrosis between the two age groups. The rate of amputation was 30% higher in the children group (P = 0.018). The rate of limb salvage surgery using reconstruction with allograft or autograft was 70% higher in the children group (P = 0.018) while endoprosthesis rate was 40% higher in the AYA group (P = 0.018). The log rank test revealed that survival is similar between the two age groups for non-metastatic patients (P = 0.424 for OS and P = 0.393 for EFS). Metastatic patients of both ages group had higher risk of dying compared to non-metastatic (HR 3.283 95% CI 2.581-4.177; P < 0.001). Children with metastases at diagnosis had less OS time (P = 0.049) and EFS time (P = 0.032) than adolescents. CONCLUSION: Non-metastatic OST in preadolescent patients does not appear to be significantly different from those seen in AYA patients, but has local control challenges. Children presenting with metastases should be considered an ultra-high-risk group.