Everton Siviero do Vale

Primeiro atendimento em queimaduras: a abordagem do dermatologista

Despite continued progress obtained recently in treating the massively burned, mortality and morbidity rates remain quite high. Implementation of preventive strategies has still not managed to significantly alter the dramatic epidemiological picture of burns patients. Survivors of severe burns still carry a heavy load of physical and psychosocial sequelae, which engenders a considerable share of suffering to overcome. There is little doubt that the final prognosis of a burn essentially depends on prompt and adequate inicial management. This is why it is important for all physicians, including dermatologists, to be able to guide first aid for and manage initially a burns victim. The aim of this article is to review the pathophysiology of burns and the principles according to which a burns victim is assessed. In addition, it is discussed a single and objective way of carrying out the emergency medical care until the victim be moved to a burns unit, if needed.

Síndrome de Muckle-Wells em quatro membros de uma família

Muckle-Wells syndrome is a rare autosomal dominant disease that belongs to a group of hereditary febrile syndromes. It is characterized by recurrent and self-limited episodes of fever, urticaria, arthralgia, myalgia and conjunctivitis since childhood, which are related to exposure to cold temperatures. Lately, progressive sensorineural hearing loss occurs. Amyloidosis is the main complication and can be found in about 25% of the cases. It has been demonstrated that there is an association with mutations in the NLRP3 gene, which codifies cryopyrin, a protein responsible for regulating the production of proinflammatory cytokines, such as interleukin-1Beta. The authors report four cases of the disease within a family.

Elastose perfurante serpiginosa em portadora da síndrome de Down

Elastosis perforans serpiginosa is a rare, primary perforating dermatosis, frequently associated with certain genetic diseases and characterized by the transepidermal extrusion of elastic fibers. The present case report describes this dermatosis in a 19-year old female patient with Downs syndrome, who presented with asymptomatic erythematous, keratotic papules in an arciform pattern, located on her right forearm and knee, which had been present for five years. Following histopathological confirmation, treatment with cryotherapy was initiated, resulting in partial remission of the lesions.

Wells syndrome associated with chronic lymphocytic leukemia

Eosinophilic cellulitis or Wells syndrome is an uncommon skin condition of unknown etiology that can occur alone or associated with other conditions. Typically, it presents with recurrent pruritic, erythematous and edematous plaques, but it can also show clinical polymorphism. Besides the cutaneous lesions, patients can experience systemic manifestations like fever, malaise, arthralgia and peripheral blood eosinophilia. We describe a case of this rare syndrome that presented with polymorphic cutaneous lesions associated with a serious systemic disease, which was revealed through the investigation of the cutaneous disease.

Disceratose congênita: relato de caso e revisão da literatura

Dyskeratosis congenita is an inherited disease characterised by the triad of abnormal skin pigmentation, nail dystrophy and mucosal leukoplakia. Non-cutaneous abnormalities (dental, gastrointestinal, genitourinary, neurological, ophthalmic, pulmonary and skeletal) have also been reported. Bone marrow failure is the main cause of early mortality, with an additional predisposition to malignancy. Men are more affected than women and X-linked recessive, autosomal dominant and autosomal recessive forms of the disease are recognised. We report a case of a male child who presented the classic triad of lesions, without familial cases. A review of the literature is also made, emphasizing the importance of a multidisciplinary approach, which is fundamental for an early diagnosis of the complications.

The consolidation of the Anais in the medical journal scenario

Completada a edição comemorativa dos 80 anos dos Anais Brasileiros de Dermatologia, o peso da maturidade remete à reflexão sobre o seu real papel na difusão do conhecimento dermatológico entre a comunidade médica brasileira e, por que não, estrangeira. Apesar da convivência com as adversidades inerentes a nações com consideráveis problemas econômicos e sociais, a Sociedade Brasileira de Dermatologia (SBD), órgão responsável por sua publicação, através de suas diretorias ao longo de todos esses anos, demonstrou constante empenho em manter o periódico ativo. No entanto, além de estabilidade, o periódico deve ter credibilidade e ser respeitado pela comunidade científica, sendo que para isso é essencial satisfazer de forma equilibrada os interesses de leitores, autores e patrocinadores. Como a revista impressa é de distribuição ampla a todos os associados da SBD, apenas a magnitude de sua tiragem 5500 exemplares não é capaz de dimensionar o real interesse dos leitores pelos artigos publicados nos Anais. Entretanto, sua disponibilização on line em versão bilíngüe permitirá em futuro próximo avaliar a evolução do número de acessos e downloads de seus artigos, o que certamente servirá de indicador do atendimento às expectativas dos leitores dos Anais. O editorial do último fascículo apresenta estatística preliminar do acesso a seus artigos, que já pode vislumbrar uma tendência de crescente interesse pelo material científico veiculado na revista, inclusive com significativo número de visitantes estrangeiros, ainda que por ora esteja disponível apenas o volume 80. Em breve, com a inclusão de todo o acervo da revista desde a sua fundação, o site dos Anais se constituirá em fonte de conhecimento dermatológico de inestimável valor, de consulta obrigatória para a elaboração de qualquer trabalho de natureza dermatológica. Por outro lado, a facilidade de acesso ao acervo dos Anais permitirá que seus artigos sejam citados em novas publicações, aumentando o fator de impacto do periódico. A ampla distribuição dos Anais aos associados da SBD, assim como o expressivo número de consultas e leituras de seus arquivos, certamente servirão de atrativo a pesquisadores e autores, que cada vez mais irão eleger os Anais como veículo para divulgação de seus trabalhos científicos. Portanto, é de se esperar que também se torne crescente o número de artigos submetidos à publicação no periódico e com isso a Equipe Editorial, o Conselho Editorial e o Conselho Consultivo terão que ser cada vez mais exigentes e rigorosos na avaliação e aceitação de trabalhos submetidos. Além disso, com o aval e o apoio da diretoria da SBD, a Equipe Editorial assumiu o compromisso de elevar o padrão de qualidade dos Anais, como pode ser percebido nas profundas mudanças instituídas ao longo dos últimos dois anos. Também essas mudanças vêm representar significante incentivo para captação de trabalhos de maior qualidade. O mercado das revistas médicas é um campo em constante expansão, embora bastante exigente e competitivo. Assim, a disputa por patrocínio é acirrada, exigindo importantes requisitos para despertar o interesse de patrocinadores e obter apoio financeiro de agências de fomento científico. Em que pese a dependência de patrocínio para a viabilidade financeira do periódico, a Equipe Editorial deve estar sempre atenta à obediência rigorosa às normas que regem a publicidade médica e farmacêutica. Enfrentando os desafios próprios desse disputado mercado, os Anais Brasileiros de Dermatologia vêm buscando se adequar à atual realidade dos periódicos científicos e para isso têm passado por constantes transformações, de modo a se afirmar como veículo de prestígio no cenário das publicações científicas no Brasil, América Latina e restante do mundo.

Thanks and reply from the editors of the ABD to the comments on the correspondence sent to heads of services accredited by SBD

Silvio Alencar Marques 0000-0002-5512-4700 We recently sent a letter to heads of services accredited by our Society with general information about the Anais Brasileiros de Dermatologia (ABD), the new section on infectious/parasitic diseases and our efforts to increase the impact factor, promote higher visibility and better classification among the main international dermatologic journals. Currently, we occupy the 54th position among the 62 dermatology journals indexed worldwide. We are ahead of Annales de Dermatologie et de Vénéréologie, and Leprosy Review. We can improve with the collaboration of all. We are among the three largest dermatology societies in the world. We would like to thank all colleagues that participated with responses and suggestions. Among the correspondences sent to the heads of dermatology services, Dr. Jaison Barreto, expressed concerns and critiques regarding the performance of the editors and the quality of the articles published, especially on Hansen’s disease, the article of Continuing Medical Education in particular.1 Dr. Laila de Laguiche, in another correspondence, criticizes the Editorial of the same journal number. In that Editorial, attention is drawn to the CME article and possibilities of changes in the therapeutic scheme for Hansen’s disease. In this number of the ABD, we transcribed the texts sent by Dr. Jaison Barreto and Dr. Laila de Laguiche, and the reply of the authors of the CME article. We believe the reply of the CME article’s authors are enough to explain the Editorial’s reasons and we will let our colleagues who work with Hansen’s disease be the judges of the quality of the articles published in our journal.

New perspectives for the treatment of Hansen's disease

In the meeting of the World Health Organization (WHO) Expert Committee on Leprosy in 1981, the need for a therapeutic regimen that could solve the growing problem of sulfone-resistant multibacillary patients and the also worrisome perspective of rifampicin and clofazimine resistance were discussed.1 From this meeting, arose the recommendation of the combination of dapsone, rifampicin and clofazimine for multibacillary and dapsone and rifampicin to paucibacillary patients. This therapeutic combination, known as Multidrug Therapy (MDT) or Polychemotherapy (PQT), changed the context of Hansen’s disease: it broke the dapsone resistance cycle and allowed for the cure of millions of patients. In view of the excellent results with PQT in the decade of 1980, in 1991 WHO launched the elimination goal for Hansen’s disease in all endemic countries until the year of 2000.2 Except for Brazil, this goal was achieved in 2005. The prevalence was reduced to the suggested rate, which is less than 1 leprosy case in 10,000 inhabitants. It was expected that, from this small number of patients in the population, the transmission would be extremely reduced, with gradual disappearance of the condition.3 However, the predictions did not materialize.3 What we see is that, despite all advances with a marked decrease in the prevalence rates (known registered cases), the incidence did not show significant differences, indicating that transmission was not significantly affected.4, 5, 6 Among the core problems for the treatment and control of Hansen’s disease are the need for a classification of the patients, adverse events of the medications and, in particular, the length of treatment: until a negative bacilloscopy is obtained in the initial phases of PQT; then a fixed duration of 24 months and, currently, 12 months. In Brazil, over the past 14 years, an important clinical essay was developed for the treatment of Hansen’s disease with a single regimen of PQT and fixed duration of 6 months, with no need for clinical classification. This study, funded by the Ministério da Saúde/CNPq, was conducted in two national tertiary dermatology centers, accredited by the Brazilian Society of Dermatology (SBD). The project was already finalized, demonstrating that the objectives are feasible and can be implemented in all endemic countries.7, 8 The use of a single therapeutic regimen, without the need for classification through tests will certainly contribute to a better operationalization of the treatment for patients; however, we still have a long way ahead to reduce the transmission and the number of new patients, with unfortunately a high proportion of deformities. In the Continuing Medical Education article of this edition we approach the different aspects related to Hansen’s disease, including the details related to treatment regimens in the past few decades.

Changes in sight

Starting its work ahead of the Brazilian Society of Dermatoloy, the new editorial board intends to implement some changes aiming mainly at improving the quality of the journal. A decision considered crucial is the standardization of the print magazine exclusively in Portuguese, since this version is directed primarily to domestic readers. Thus, the national authors and foreigners who have Portuguese as their native language must submit their work in Portuguese, being given them the option to attach a translation of the article in English. The studies of foreign authors of other languages should be submitted in English; these studies, however, will be translated into Portuguese, wich will be afforded by the magazine, to be published in the printed journal. Thus, the printed magazine will have a uniform standard in a single language, attending the interests of all national readers, including even who does not master reading in English. However, the online version of the journal will remain in English, attending the interest of foreign readers and ensuring international visibility for the journal. Another change will be the return of the section “What’s your diagnosis”, considering the importance of image in the formulation of the diagnosis in this specialty. This section is intended, therefore, to provide a brief report of a case of undeniable interest, illustrated by clinical imaging, and usually also by complementary examination, especially histopathology, in which the final diagnosis of the clinical presentation is questioned, followed by a brief discussion of the disease. It is still important to highlight the limitation of the number of authors according to the nature of the article. Based on strict criteria of authorship contained in the guidelines of the journal, the maximum number of four authors was considered sufficient for the different sections of the journal, except for investigation and review articles, which will be limited to six authors. However, exceptionally, editors may admit a larger number of authors, when the complexity of the study justifies it. With implications in the impact factor, dependent on citations of articles published in the magazine, it becomes forcible to raise the rigor in the selection criteria of the articles, especially clinical cases, for which editors will be more attentive regarding quality. Thus, reports of cases considered simpler should be directed to the section “Letter to the Editor”, which will have more limited space for text, illustrations and references. Finally, in order to speed the publication process and make less burdensome the task of the reviewers, the new editors appeal to the authors to put great care in the preparation of their manuscripts, with close attention to the correct use of the language, to the publishing standards of the journal and to the technical quality of the illustrations.

Reflecting about frustrations and potentialities of the Anais

“importância” da revista foi de 3,5 – portanto, inferior ao mínimo necessário. A não indexação na MEDLINE nos obriga, todos, a várias reflexões. A primeira delas é no sentido de que os esforços voltados para a melhoria da qualidade científica da dermatologia brasileira sejam redobrados e que os investimentos na capacitação e qualificação dos serviços tenham mais ênfase. E, ainda, que as exigências, com vistas a credenciamento, ocupem patamares mais altos do que os vigentes. A todos, mas particularmente à Editoria Técnica e Cientifica, ao Conselho Editorial e ao Conselho Consultivo dos Anais Brasileiros de Dermatologia, cabe assumir o compromisso de máxima exigência com a qualidade técnicocientífica dos artigos submetidos e reafirmar a confiança nos destinos do periódico que representa a dermatologia brasileira. Ainda que sujeito a críticas e a alguns vieses de análise, o “fator de impacto” de um periódico tem sido considerado parâmetro de sua importância ou relevância no universo científico. Também nesse aspecto os Anais têm obtido pontuações abaixo de suas possibilidades e méritos. Recentemente chamamos a atenção, no Editorial do número 81(5), para artigo publicado no Braz J Med Biol Res. 2006;39:997-1002, que discute a tendência do autor brasileiro a citar artigos publicados em revistas internacionais, preterindo aqueles publicados em revistas editadas no país. Os Anais Brasileiros de Dermatologia, assim como outros periódicos nacionais, se ressentem dessa prática, que se reflete em “fator de impacto”, calculado pela SciELO, incompatível com nossas potencialidades e aspirações. Essas considerações remetem a profundas reflexões e discussões, que deverão ter lugar, particularmente, no âmbito dos 62 Serviços Credenciados pela Sociedade Brasileira de Dermatologia. A Editoria Científica dos Anais Brasileiros de Dermatologia volta a chamar a atenção para os acréscimos e detalhamentos efetuados nas “Instruções aos Colaboradores”, divulgadas em encarte no fascículo número 1 do volume 82 da revista e tambémdisponíveis no endereço www.anaisdedermatologia.org.br. Recomenda-se sempre a leitura atenta das informações ali contidas antes de se iniciar a redação do texto científico. Inúmeros atrasos e eventuais desencontros observados entre a Editoria Técnica e o corpo de colaboradores poderiam ser evitados pelo devido conhecimento e rigoroso respeito às instruções. Vale destacar que as normas são elaboradas com a finalidade não apenas de ressaltar a qualidade intrínseca do texto e de suas ilustrações, mas igualmente de definir a personalidade da revista e atender a normas editoriais internacionais. Como divulgado, os Anais não atingiram o principal objetivo do ano de 2006, ou seja, a indexação na base de dados MEDLINE, da National Library of Medicine. Os avanços, melhorias e cuidados técnicos não foram considerados suficientes pelo comitê de avaliação. Na sistemática de pontuação utilizada, com valores variando entre 0 e 5, e média mínima necessária de 4 para ter recomendada a indexação, os Anais Brasileiros de Dermatologia obtiveram média 3,5. Quanto ao quesito “qualidade”, os itens mais bem avaliados, considerados excelentes e correspondendo à nota 4, foram os artigos de revisão, os relatos de caso e a qualidade gráfica da revista. A média final no quesito “qualidade”, levando-se em conta o mérito científico, foi de 3,5. No quesito “importância” da revista, as melhores notas (nível muito alto, equivalendo à nota 4) foram obtidas nos itens relevância para clínicos da própria área e importância para a política local de saúde com ênfase na nosologia regional. A média final no quesito