Do you know this syndrome? Clouston syndrome

Abstract Ectodermal dysplasias are conditions that present primary defects in two or more tissues of ectodermal origin and can be classified as hypohidrotic and hidrotic. Hidrotic ectodermal dysplasia or Clouston syndrome is an autosomal dominant genodermatosis and appears as a triad of clinical findings: palmoplantar keratoderma, nail dystrophy, and hypotrichosis. The hair is sparse and brittle. The nails become thickened and dystrophic, which is an essential characteristic of the syndrome. The diagnosis is made based on clinical findings. This study reports a case of a patient who began with changes in hair, nails and palmoplantar keratoderma in early childhood.

Aumento do volume labial com o uso de toxina botulínica / Lip volumization using botulinum toxin

Introdução: A aplicação da toxina botulínica na região perioral é procedimento minimamente invasivo que permite melhora substancial nos sinais de envelhecimento e aumento do volume dos lábios, com pouca documentação ainda na literatura. Objetivo: Avaliar alterações na forma e volume dos lábios com aplicações de toxina botulínica nas linhas periorais bem como a satisfação do paciente. Métodos: 19 pacientes submeteram-se a análise, registro fotográfico e medições seguidos de aplicação de toxina botulínica na região perioral. Posteriormente, foram avaliados quanto a alterações labiais e satisfação. Resultados: A maioria dos pacientes considerou os lábios moderada ou significativamente mais atraentes após a toxina (p = 0,039), e 15 mulheres notaram a mudança do lábio como um dos principais contribuintes para a melhoria global da face. Conclusões: A aplicação de toxina botulínica na região perioral provoca elevação do lábio superior, levando ao encurtamento do filtro e extensão do vermelhão. Ao aplicar a toxina na borda do vermelhão, permitimos o relaxamento do músculo orbicular, favorecendo maior efeito do tônus muscular dos músculos de elevação do lábio superior. A satisfação das pacientes com a aparência dos lábios avaliada pelas quatro perguntas adicionais (forma, volume, atração e beleza) foi positiva, com efeitos colaterais mínimos.

Introduction: The application of botulinum toxin in the perioral region is a minimally invasive procedure that leads to substantial improvement in the signs of aging in addition to increasing the volume of the lips. However literature on this procedure is still scarce. Objective: To evaluate changes in shape and volume of the lips, as well as the patients' satisfaction after the application of botulinum toxin in perioral lines. Methods: Nineteen patients underwent evaluation, photographic record and measurements following the application of botulinum toxin in the perioral region, being subsequently assessed for changes in the lips and satisfaction. Results: Most of the patients rated their lips as moderately or significantly more attractive after the application of botulinum toxin (p = 0.039). Fifteen women deemed the changes in the lips as a major factor in the overall improvement of their faces. Conclusions: The use of botulinum toxin in the perioral region leads to the elevation of the upper lip, causing the shortening of the philtrum and the extension of the vermilion. The application of botulinum toxin in the vermilion's border leads to the relaxation of the orbicularis muscle, favoring a more intense tonus effect arising from the upper lip lifting muscles. The patients' satisfaction with the appearance of their lips was evaluated by four additional questions (linked to the lip's shape, volume, attraction and beauty), receiving positive answers. Side effects were minimal.

Case for diagnosis. Lichen myxedematosus

Abstract Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy.

Syndrome in question

Abstract: Morbihan Syndrome is a rare entity with unknown etiology. It is clinically characterized by chronic erythematous edema on the face - especially in the middle and upper third of the face - and creates abnormal facial contours that are initially intermitent but become permanent with the development of the syndrome. The histopathology is nonspecific and its therapy is a major challenge due to poor response to the various treatment options. We present the case of a male patient with a five-month-history of disease.

Manifestações dermatológicas em pacientes infectados pelo HIV: um estudo de prevalência / Dermatological manifestations in HIV-infected patients: a prevalence study

Na infecção pelo HIV, a pele pode ser tanto um indicador precoce de doença quanto de gravidade. Este estudo analisou a frequência e a apresentação clínica de manifestações dermatológicas em pacientes portadores de HIV, destacando as lesões mais encontradas, e correlacionou suas características com o estado imunológico. Foram encontradas 129 lesões mucocutâneas dentre os 57 pacientes portadores de HIV examinados, havendo em média 2,25 lesões por paciente. As dermatoses mais comuns, em ordem decrescente de frequência, foram candidíase oral, onicomicose e dermatite seborreica. As lesões oportunistas mostraram relação significativa com deficiência imunológica, e as afecções de pele mais encontradas mostraram-se bons indicadores do estado imunológico do paciente e da progressão da doença.

In the HIV infection the skin can be either an early indicator of disease and severity. This study examined the frequency and clinical presentation of skin manifestations in patients with HIV, highlighting the most frequent injuries, and correlated their characteristics with the immune status. We found 129 mucocutaneous lesions among 57 patients with HIV, with an average of 2.25 lesions per patient. The most common skin diseases in descending order of frequency were oral candidiasis, onychomycosis and seborrheic dermatitis. Opportunistic lesions showed significant relationship with immune deficiency and the most frequent skin diseases proved to be good indicators of the immune status of the patient and disease progression.

Reativação de tuberculose no tratamento com imunobiológicos / Reactivation of tuberculosis in treatment with immunobiological

O uso de agentes biológicos vem se mostrando uma boa opção no tratamento da psoríase de difícil controle. Os inibidores do fator de necrose tumoral alfa (TNF-alfa) demonstraram resultados positivos tanto em índices de resposta terapêutica quanto em velocidade de início de ação. No entanto, pelo fato de o TNF-alfa ter uma importante participação na formação do granuloma e, consequentemente na defesa contra o Mycobacterium tuberculosis, tal tratamento pode resultar na reativação de doença latente. Assim sendo, o screening para tuberculose é necessário antes e durante o uso destas drogas na prática clínica.

The use of biologics agents has been a good option in the trteatment of resistant psoriasis. The tumor necrosis factor-alpha (TNF-alpha) blockers demonstrated positives results, both in efficacy and onset of action. However TNF-alpha plays an important role in host defense against tuberculosis, this treatment can result in reactivation of latent disease. Thus, screening for tuberculosis is necessary before and during the use of these drugs in clinical practice.