Eruptive Junctional Nevi Appearing During Langerhans Cell Histiocytosis Treatment.

Langerhans cell histiocytosis (LCH) is a multisystemic disorder that results from the clonal proliferation of immunophenotypically and functionally immature Langerhans cells (LC). The detection of the V600E mutation in the BRAF oncogene in LCH biopsy specimens supports previous evidence that LCH is a neoplastic disorder. This mutation is present in other cutaneous lesions including malignant melanoma and benign nevi. Single case reports of a correlation between LCH and the appearance of eruptive nevi limited to the inguinal folds after chemotherapy have previously been described in the literature. This suggested that LCH could be an additional cause of eruptive melanocytic nevi, with a specific distribution mimicking that of LCH cutaneous lesions. We present the case of a 6-year-old boy, previously treated with chemotherapy for Langerhans cell histiocytosis, with disseminated junctional nevi. Although this co-occurrence may be coincidental, the skin involvement is distinct from other previously reported clinical cases. It would be interesting to evaluate whether the BRAF mutation described in LCH cells might in fact support a genetic background for the development of nevi in these patients. LEARNING POINTS: Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of immature Langerhans cells, with the V600E mutation in the BRAF oncogene present in approximately 60% of cases.The V600E mutation in the BRAF oncogene is also documented in other cutaneous lesions, namely malignant melanoma and benign nevi.There are case reports of a correlation between LCH and the appearance of eruptive nevi after chemotherapy, but it is not known whether the BRAF mutation described in LCH cells supports a genetic background for the development of nevi in these patients.

A case of chronic and relapsing paget disease of the vulva

Abstract Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.

An Unusual Presentation of Metastatic Breast Carcinoma.

Cutaneous metastasis from a carcinoma is a relatively uncommon phenomenon. Prompt diagnosis is crucial, as it will have future implications, particularly regarding prognosis and treatment. Skin metastases can be suspected and recognized earlier through physical examination than metastases in other organs or systems. However, they can be a diagnostic challenge due to the variable clinical presentation. This case highlights the importance of having a high index of suspicion for cutaneous metastases, especially in patients with a previous history of cancer. LEARNING POINTS: Cutaneous metastases occur in approximately 0.7-10% of patients with invasive carcinomas and, when present, usually indicate an unfavourable outcome.Clinically, cutaneous metastasis can pose a diagnostic challenge and a high index of suspicion is mandatory for a prompt diagnosis.Skin biopsy of such lesions is important to confirm the diagnosis and, in the right clinical scenario, they can provide information on the primary origin of the tumour.

A Case of Chronic and Relapsing Paget Disease of the Vulva.

Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.

[Career Satisfaction of Medical Residents in Portugal]. / Satisfação com a Especialidade entre os Internos da Formação Específica em Portugal.

INTRODUCTION: The satisfaction with the medical profession has been identified as an essential factor for the quality of care, the wellbeing of patients and the healthcare systems' stability. Recent studies have emphasized a growing discontent of physicians, mainly as a result of changes in labor relations. OBJECTIVES: To assess the perception of Portuguese medical residents about: correspondence of residency with previous expectations; degree of satisfaction with the specialty, profession and place of training; reasons for dissatisfaction; opinion regarding clinical practice in Portugal and emigration intents. MATERIAL AND METHODS: Cross-sectional study. Data collection was conducted through the "Satisfaction with Specialization Survey", created in an online platform, designed for this purpose, between May and August 2014. RESULTS: From a total population of 5788 medical residents, 804 (12.25 %) responses were obtained. From this sample, 77% of the responses were from residents in the first three years. Results showed that 90% of the residents are satisfied with their specialty, 85% with the medical profession and 86% with their place of training. Nevertheless, results showed a decrease in satisfaction over the final years of residency. The overall assessment of the clinical practice scenario in Portugal was negative and 65% of residents have plans to emigrate after completing their residency. CONCLUSION: Portuguese residents revealed high satisfaction levels regarding their profession. However, their views on Portuguese clinical practice and the results concerning the intent to emigrate highlight the need to take steps to reverse this scenario.

Introdução: A satisfação com a profissão médica tem sido apontada como um fator essencial para a qualidade assistencial, o bemestar dos doentes e a estabilidade dos sistemas de saúde. Estudos recentes têm vindo a enfatizar um crescente descontentamento dos médicos, principalmente como consequência das alterações das relações laborais.Objetivos: Avaliar a perceção dos médicos de formação específica em Portugal, sobre as expectativas e grau de satisfação com a profissão, especialidade e local de formação; razões da insatisfação e intenção de emigrar.Material e Métodos: Estudo transversal. A colheita de dados foi efetuada entre Maio e Agosto de 2014 através de um Inquérito online sobre a âÄúSatisfação com a EspecialidadeâÄù.Resultados: De uma população total de 5788 médicos, foram obtidas 804 respostas (12,25% do total de médicos internos). Desta amostra, 77% das respostas correspondem a internos dos três primeiros anos de formação. Verificou-se que 90% dos médicos se encontram satisfeitos com a especialidade, tendo-se encontrado também níveis elevados de satisfação com a profissão (85%) e local de formação (86%). Por outro lado, constatou-se que estes diminuíam com a progressão ao longo dos anos de internato. A avaliação global sobre o panorama da prática médica foi negativa e 65% dos médicos responderam que consideram emigrar após conclusão do internato.Conclusão: Os médicos internos em Portugal apresentam níveis positivos de satisfação com a sua profissão. No entanto, a sua opinião sobre o panorama da Medicina e os resultados relativos à intenção de emigrar alertam para a necessidade de tomada de medidas para inverter este cenário.