Basal cell carcinoma (BCC) is the most common skin cancer. It generally has an indolent course with low rates of metastasis and mortality. However, BCC is locally invasive and can cause significant morbidity due to destructive local spread. We report our experience with a patient who was referred to our skin cancer unit due to a previously neglected lesion on the parietal region of the scalp, which had developed for 7 years. The patient was prescribed vismodegib on the basis that surgery could cause excessive functional and aesthetic damage. The patient had an objective partial response after 20 months of treatment. He was then submitted to radical skin excision, leaving a large defect that was reconstructed using a free latissimus dorsi muscle flap. The patient recovered well, and at the 1-year follow-up there were no signs of local recurrence.Our case demonstrates the value of vismodegib treatment prior to surgery in a locally advanced, high-risk scalp BCC and highlights the importance of an individualized and specialized approach with these patients, within a multidisciplinary team.

Malignant melanoma (MM) arising primarily in the cervix is exceedingly rare and has a poor prognosis. We report the case of a primary MM of the cervix in a 64-year-old woman with vaginal bleeding. She presented with a cervical amelanotic lesion which on biopsy rendered the diagnosis of MM. The patient was staged as International Federation of Gynecology and Obstetrics IIB and underwent Wertheim-Meigshysterectomy followed by brachytherapy. One year later, she was diagnosed with a large pelvic relapse for which surgery was performed. She then presented with a vaginal relapse and an isolated hepatic lesion, both of which were proposed for surgery. The diagnosis of MM of the cervix is a clinical and pathological challenge due to its rarity and overlapping features. Cytology cannot accurately diagnose it. Moreover, amelanotic MMs must be distinguished from other poorly differentiated carcinomas by diagnosis that ultimately relies on immunohistochemical staining. Radical surgery is the only treatment showing predictive benefit.

No abstract

Eccrine hidradenocarcinoma is a rare adnexal neoplasm arising from the eccrine sweat glands of the skin. Surgery and radiotherapy are the mainstay of treatment, and chemotherapy is reserved for unresectable or metastatic lesions. We present the case of a 60-year-old man, referred for treatment of an unresectable basal cell carcinoma of the scalp. He started Vismodegib in January of 2017 but progressed after three months. A new biopsy showed a poorly differentiated carcinoma. The patient started carboplatin/paclitaxel with a major response, enabling surgery in December of 2017. Pathology concluded on a hidradenocarcinoma, R1, and radiotherapy was not possible due to local infection. Four months later, he underwent radiotherapy due to local recurrence, and restarted carboplatin/paclitaxel, but with progressive disease. An exploratory surgery in October of 2018 revealed unresectable disease. Restaging showed lung metastasis and second-line chemotherapy was proposed. However, due to continued clinical deterioration, the patient died in February of 2019.

No abstract

ResumoOs autores apresentam o caso clínico de uma doente de 79 anos, internada para estudo de uma vasculite, cuja investigação etiológica nos levou a ponderar a associação com hepatite B crónica. A vasculite leucocitoclás-tica pode ter variadas etiologias. A associação com hepatite vírica é mais frequente com a hepatite C, havendo no entanto alguns casos relatados sobre a associação à hepatite B. A etiologia vírica tem de ser sempre considerada nestas situações, pois a resolução do quadro pode passar pelo tratamento da patologia de base.Palavras chave: Vasculitis leucocitoclàstica. Hepatitis B crònica. Crioglobulinas. AbstractThe authors present a case report of a 79 year old patient who was admitted for the study of vasculitis, whose etiological investigation led us to consider the association with chronic hepatitis B infection. Leucocytoclastic vasculitis may have varied etiologies. The association with viral hepatitis is more common with hepatitis C, but there are some cases reported about the association with hepatitis B. The viral etiology must always be considered in such situations because the case resolution can implicate the treatment of the underlying disease.

ResumoConsidera-se a presença do síndrome em pacientes com anticorpos antisintetase associados a duas das seguintes características, doença pulmonar intersticial, miopatia inflamatória e poliarterite inflamatória.Apresentamos o caso de um homem com diagnóstico efectuado há 13 anos, durante um internamento por "pneumonia", apresentando queixas de dispneia para esforços e cansaço fácil com 3 meses de evolução. Durante o internamento é identificada uma doença pulmonar intersticial e posteriormente surge um quadro de mialgias com elevação marcada das enzimas musculares. A electromiografia mostrou um padrão sugestivo de miopatia e identificou-se anticorpos anti-Jo-1, estabelecendo-se o diagnóstico de síndrome anti-sintetase. Sob terapêutica imunossupressora desde então, mantém seguimento em consulta externa, encontrando-se clinicamente bem.Palavras chave: síndrome antisintetasa; enfermedad pulmonar intersticial; miositis; anti-Jo-1. AbstractThe syndrome is generally considered present in patients with an antisynthetase antibody plus two of the following features, interstitial lung disease, inflammatory myopathy, and inflammatory polyarthritis.We report the case of a man with diagnosis made 13 years ago, during hospitalization for "pneumonia" with complaints of effort dyspnea and tiredness with 3 months of evolution. Non infectious interstititial lung disease was finally demonstrated and subsequently myalgia occurs with a marked elevation of muscle enzymes. Electromyography shows a pattern suggestive of myopathy and with identification of anti-Jo-1 the diagnosis of anti-synthetase syndrome is established. The patient is on immunosuppressive treatment since then, being followed at the outpatient clinic, without symptoms at the present moment.Keywords: anti-synthetase syndrome; interstitial lung disease; anti-Jo-1; myositis. IntroduçãoO síndrome anti-sintetase caracteriza-se pela associação de duas das seguintes características, doença pulmonar intersticial (DPI), miopatia inflamatória e poliarterite inflamatória com a presença de anticorpos anti-sintetase. Laboratorialmente é marcada pela presença de anticorpos anti-aminoacil-RNAt sintetase (anti-sintetase), sendo mais frequente o anti-Jo-1 1 . Trata-se de uma doença pouco comum, com uma prevalência de 1,5 por 100.000 habitantes 2 , tendo sido descrita em 1990 por Marguerie C, et al. Caso ClínicoApresentamos o caso de um homem, de 65 anos, não fumador, com antecedentes de hiperplasia benigna da próstata e doença coronária, que apresentava queixas de dor pré-cordial, cansaço fácil e dispneia para pequenos esforços com 3 meses de evolução associado nas duas ultiamas semanas a sudorese nocturna e tosse produtiva. Recorreu à consulta de Cardiologia (em Junho de 2000) tendo realizado prova de esforço e ecocardiograma que não mostraram alterações. Posteriormente recorreu ao Serviço de Urgência (SU), onde no estudo realizado se constatou a presença no RX de tórax de opacidades heterogéneas de ambas as bases e hipoxémia (pO 2 de 69,1mmHg) na gasimetria arterial. Este quadro f...

No abstract