No abstract

Morbihan syndrome is a rare entity that more commonly affects women in the third or fourth decade of life. It is considered a special form of rosacea and its pathogenesis is not fully known. It is clinically characterized by the slow appearance of erythema and solid edemas on the upper portion of the face, with accentuation in the periorbital region, forehead, glabella, nose, and cheeks. We report the case of a patient presented with edema on the upper eyelid for a year. These findings suggested the diagnosis of Morbihan syndrome. We aim to report a rare, particularly refractory and chronic form of rosacea, which has received little attention in the literature.

Plasmoacanthoma is an extremely rare verrucous tumor located on periorificial regions characterized by dense dermal plasmacytic infiltrates. Some authors classify it as a form of reactive plasma cell proliferation which represents a heterogeneous spectrum of mucocutaneous disorders. These plasma cell proliferations have been considered to be a benign immunologic inflammatory reaction to known or unknown stimuli. However, the etiology of plasmoacanthoma remains highly speculative. We report the case of a 40-year-old woman who presented with a lobulated warty lesion affecting the lower lip. Biopsy from the lesion was compatible with plasmoacanthoma, which remains an underreported disease in the dermatology literature.

BACKGROUND: Lymphadenitis is common in HIV-positive patients. Diagnosis of the infections associated with this condition is complex, particularly in the case of tuberculosis. Rapid and specific detection of Mycobacterium tuberculosis (M. tuberculosis) is fundamental in ensuring adequate treatment. In addition, frequent causes of lymphadenitis such as those associated with lymphoma and histoplasmosis, among others, must be eliminated as possible causes. OBJECTIVES: To evaluate the accuracy of polymerase chain reaction as a tool for the diagnosis of lymphadenitis resulting from M. tuberculosis. METHODS: In this study, a protocol was developed using the following procedures: direct microscopy using Ziehl-Neelsen staining, culture in Lowenstein-Jensen medium, histology and polymerase chain reaction. RESULTS: A total of 104 patients were included in the study. According to histopathology, 38 patients (36%) were found to have nonspecific chronic lymphadenitis, 27 (26%) had tuberculous lymphadenitis, 11 patients (10.5%) had lymphoma and 9 (8.7%) had histoplasmosis. When Lowenstein-Jensen culture was performed, positive tests for tuberculous lymphadenitis increased by 30%. With polymerase chain reaction, M. tuberculosis DNA was detected in 6 out of 38 samples of non-specific chronic lymphadenitis. Three of these patients were followed up, developed symptoms of tuberculosis and were cured following specific treatment. CONCLUSION: The data obtained in this study suggest that all cases of lymphadenopathies should be submitted to histopathology, Lowenstein-Jensen or Ogawa culture and polymerase chain reaction. Polymerase chain reaction may prove to be useful in providing an early and accurate detection of cases of extrapulmonary tuberculosis in HIV-positive patients with lymphadenopathies, avoiding empirical treatment and the possible development of resistant strains.

Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous-violaceous lesions that leave no scars after regression. Histopathology reveals perivascular and periannexal lymphohistiocytic infiltrates in the papillary and reticular dermis and interstitial mucin deposition. Treatment is based on photoprotection, topical corticosteroids and antimalarials. We report two cases of lupus tumidus, which deserve attention for their low frequency in the literature, in addition to their relevance as a differential diagnosis among dermatologic disorders.

Background The primary objective of this study was to evaluate the demographic, clinical, social-educational determinants and diabetes management factors that have influenced the agreement between glycaemia obtained from a glucometer and logbook; the second objective was to evaluate the influence of the above-mentioned factors on glycemic control and its trajectories in Type 1 diabetes (T1D) over 1 year follow-up period during routine clinical practice. Methods This was a prospective observational cohort study conducted at the Diabetes Unit at Rio de Janeiro’s State University, between May 2017 and May 2018. All consecutive patients with clinical diagnosis of T1D that attended the Diabetes Unit between April and June 2017 were enrolled in this study. Results Data were obtained from 158 patients. Overall, for 112 (73.2%) of the patients, we found no agreement between glycaemia obtained from a glucometer and the logbook (group 2). In 41 (26.8%) of the patients there was an agreement (group 1). Patients from group 1 presented a lower mean glycated hemoglobin (HbA1c) (p = 0.03) and a tendency to have a lower baseline HbA1c (p = 0.08), they received more frequently strips for glucose monitoring from the Sistema Único de Saúde (SUS) (p = 0.047) and were more adherent to the prescribed diet (p = 0.01) than patients from group 2. Multivariate analysis of this agreement (as a dependent variable) showed that adherence to diet was the only significant independent variable. Significant difference was noted between baseline and final HbA1c [(9.4 ± 2.2%) vs (9.03 ± 1.8%), p = 0.017], respectively. Conclusions Our study revealed that the majority of T1D patients that were followed at a tertiary center did not have significant agreement between glycaemia obtained from a glucometer and a logbook. Adherence to diet was the main factor related to the agreement, but the supply of strips by SUS should also be considered in clinical practice.

INTRODUCTION: Leucinosis or Maple Syrup Disease (MSD) is an innate metabolism error caused by a deficiency in the activity of the alpha-keto acid-dehydrogenase enzyme complex, which leads to the accumulation of essential branched-chain amino acids (leucine, valine, and isoleucine), leucine is particularly toxic to the central nervous system (CNS). OBJECTIVES: Report a case of maple syrup disease in a 10-day-old newborn (NB), born to consanguineous parents, with late diagnosis, with high levels of leucine, with an unfavorable prognosis. COMMENTS: MSD is a rare condition, of autosomal recessive origin, which occurs due to the accumulation of essential branched-chain amino acids in tissues(leucine, valine, and isoleucine). The accumulation of amino acids mainly affects the CNS. Studies have shown that the elevated leucine concentration decreases the serum sodium concentration and increases intracellular water, causing cerebral edema. Neurological damage will depend on the degree and time of tissue exposure to metabolites. Despite the unfavorable outcome of the case presented, the prognosis of MSD can be modified with strict diet control and early and aggressive management of metabolic crisis.

This article discusses about the idealization and the Teaching Hand prototype development process. This software will be a platform dedicated to the teaching-learning process of Portuguese, as a second language, for deaf children. The teaching-learning methods for deaf people encounter great difficulties due to problems in acquiring Portuguese as L2. The main difficulties do not stem from deafness itself, but mainly the lack of rapprochement with the Portuguese. For this reason, the Teaching Hand demand offer the best educational conditions for creating an educational environment that encourages learning and enables the integration of the deaf in the form of equality to listeners. Resumo.O presente artigo discorre sobre a idealização e o processo de desenvolvimento do protótipo do Teaching Hand. Este software será uma plataforma voltada ao processo de ensino/aprendizagem da Língua Portuguesa, como segunda língua, por crianças surdas inseridas nos níveis fundamentais I e II. Os métodos de ensino-aprendizagem para sujeitos surdos encontram grandes empecilhos devido a problemas na aquisição da Língua Portuguesa como L2. As principais dificuldades não decorrem da surdez em si, mas, principalmente, da falta de aproximação com a Língua Portuguesa. Por esse motivo, o Teaching Hand procura ofertar as melhores condições pedagógicas para criação um ambiente educacional que estimule o aprendizado e possibilite a inserção do surdo em forma de igualdade aos ouvintes. Cenário de UsoEstudos recentes no âmbito linguístico têm apontado um crescente reconhecimento da linguagem enquanto lugar de interação. Entretanto, para uma grande maioria de sujeitos surdos, esta é uma realidade ainda distante.Por muito tempo, o surdo viveu à margem da sociedade e, em se tratando de linguagem, não dispunha de um método específico voltado para auxiliá-lo em suas limitações comunicacionais. No decorrer dos séculos, no entanto, tem-se proposto uma