Journal of the Portuguese Society of Dermatology and Venereology
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Genes e Melanoma
Get PDFMajor advances have been made in the understanding of melanoma in the past decade. The pathophysiology of genetic aberrations in melanoma oncogenesis as well as the evidence of family risk associated, have relevant implications in thetreatment and prognosis of patients, namely in patients with advanced disease.In this paper the authors describe, in a schematic and precise way, the different molecular pathways implicated in oncogenetic events in melanoma, which are the molecular targets of new on-going and newly developed targeted therapies.A compreensão do desenvolvimento do melanoma tem sido alvo de avanços importantes na última década. A relevância fisiopatológica das alterações genéticas na oncogénese do melanoma bem como a evidência do risco familiar associado têm condicionado de forma positiva o tratamento e prognóstico dos doentes, nomeadamente nos doentes com doença avançada.Neste artigo os autores descrevem de forma sucinta e esquemática as diferentes vias de sinalização celular, com importância na oncogénese do melanoma e que se encontram subjacentes à génese dos novos alvos terapêuticos presentemente em utilização e em desenvolvimento
Infiltração Cutâneo-Mucosa Perioral / Perioral mucocutaneous infiltration
Get PDF.Apresenta-se o caso de uma mulher de 75 anos, caucasiana, com achados clínicos compatíveis com sialadenite esclerosante crónica e discutem-se os achados laboratoriais, histológicos e radiológicos que permitiram estabelecer o diagnóstico de uma Doença relacionada com IgG4. Discutem-se os critérios de diagnóstico, tratamento e evolução clínca da doente
Resposta Imunológica no Melanoma: Base para a Compreensão do Papel da Imunoterapia com Inibidores de “Checkpoints” Imunológicos
Get PDFThe knowledge of the pathophysiology of tumour progression is crucial to understand the therapeutic targets in order to control the disease. The mechanisms used by the immune system to affect cancer development and progression has been a challenging question in immunology. It is now postulated that immunology plays a dual role in this process: it protects against tumour growth, destroying “aberrant” tumour cells, but may also promote tumour progression by selecting tumour cells that are able to escape the immune response and survive in an immunocompetent host. These findings gave rise to the concept of “cancer immunoediting”, which explains the influence of the immune system on tumour progression. Several observations like immunosuppression as a risk factor for melanoma, the possibility of partial or complete regression of primary tumour and development of vitiligo, have suggested that melanoma is an immunogenic tumour but a successful tumour evolution can occur in the light of the “immunoediting” concept. Immune checkpoints, cytotoxic T lymphocyte antigen (CTLA)-4 and programmed cell death (PD-1), were recognized to have important roles in regulating T cell responses during tumour development and were proven to be effective targets in treating advanced melanoma. This article will briefly review the process of tumour evolution and its interaction with the immune system as well as the mechanism of action of the immune checkpoint inhibitors to understand better the new targeted immunotherapies for advanced melanoma, that will be further discussed.O conhecimento do processo de evolução tumoral é essencial para compreender os alvos terapêuticos no controle da doença. A forma como o sistema imune influência o desenvolvimento e a progressão do cancro é uma questão desafiante na área da imunologia. Atualmente reconhece-se o papel paradoxal do sistema imunológico neste processo: por um lado protege contra o crescimento tumoral, destruindo células exprimindo antigénios tumorais “aberrantes”, por outro pode favorecer a sua progressão, selecionando células tumorais que escapam à vigilância imunológica e são capazes de sobreviver num hospedeiro imunocompetente. Esta observação deu origem ao conceito de “cancer immunoediting”, que explica a influência do sistema imune na progressão tumoral. Tendo em conta algumas observações associadas ao melanoma, como por exemplo, o desenvolvimento de vitiligo, a possibilidade de regressão e a correlação com a imunossupressão, este tem sido considerado um exemplo de tumor imunogénico, cujo mecanismo patofisiológico reconhecido até à data se enquadra no conceito de “immunoediting”. Reconhecida a importância de CTLA-4 (antigénio linfócitário T citotóxico) e PD-1 (proteína de morte celular programada) como “checkpoints” imunológicos na regulação da atividade das células T em resposta à progressão tumoral, estas moléculas têm sido considerados alvos terapêuticos importantes no tratamento do melanoma avançado. O presente artigo pretende rever sucintamente o processo de evolução tumoral e respetiva interação com o sistema imune, bem como o mecanismo de ação dos “checkpoints” inibitórios por forma a melhor compreender os novos alvos da imunoterapia no melanoma avançado, que serão revistos em trabalho futuro
Patch and Photo-Patch Testing are Important in Patients with Idiopathic Photodermatoses
Get PDFThis number of the Revista da Sociedade Portuguesa de Dermatologia e Venereologia contains two articles dedicated to idiopathic photodermatoses, for which autoimmune reactions to an unknown endogenous chromophore are suspected to be involved – polymorphous light eruption, actinic prurigo, hydroa vacciniforme, chronic actinic dermatitis, and solar urticarial.1,2 Many of these and other photodermatoses have a very clear clinical presentation, while others may mimic allergic contact dermatitis (ACD) or photo-allergic contact dermatitis (PhACD), a classical T cell-mediated or delayed type IV hypersensitivity reaction to an exogenous chromophore applied on the skin in the presence of, or followed by exposure to ultraviolet (UV) or visible light.3,4 Allergic contact reactions can be followed by persistent photosensitivity and chronic actinic dermatitis, such as in cases of chronic ACD from certain plants, e.g., Compositae that are rich in sesquiterpene lactones,5 fragrances, lichens, and colophony,4 or in PhACD or photo-aggravated ACD from drugs like ketoprofen, etofenamate, and chlorproethazine, or even other contact allergens, such as tosylamide/formaldehyde resin, fragrances, and thiourea derivatives.4The long persistence of these chemicals in the epidermis (for up to at least 17 days in the case of ketoprofen),6 or the formation of endogenous photosensitizers might perhaps explain the progression to chronic actinic dermatitis.4In patients with idiopathic photodermatoses the use of sunscreens is mandatory, however, the sensitization risk from these chemicals may be enhanced by the previous skin inflammation and the need for repeated application for long periods.7 UV filters, which are chromophores that capture UV light, are among the most frequent causes of PhACD,8-11 namely benzophenones, dibenzoylmethane derivatives, octocrylene, and cinammates.9,10,12-14 Although more recent UV filters seem to be more photostable and less prone to induce PhACD,3 a few cases have been described,9 for example, from polysilicone-15 (Parsol®SLX).15 With regard to methylene bis-benzotriazolyl tetramethylbutylphenol (syn. bisoctrizole or Tinosorb® M), ACD from it is due to the surfactant decyl glucoside, in particular, which is added in order to stabilize the sunscreen molecule.16,17Topical drugs, such as the non-steroidal anti-inflammatory ketoprofen, piketoprofen, suprofen, etofenamate, piroxicam, and benzydamine,18 as well as phenothiazine derivatives, i.e., promethazine or chlorproethazine, and isothipendyl chlorhydrate19 are frequent causes of ACD/PhACD, either by direct application or by transfer from other individuals in close contact (consort or connubial dermatitis). Moreover, some of these chemicals, particularly ketoprofen, exhibit cross-reactions with UV filters, i.e., benzophenone(s) and octocrylene, the latter containing benzophenone residues. Also fenofibrate, a systemic drug, shares the benzophenone ring and can cross react with ketoprofen and related molecules.3,20 Furthermore, patients with PhACD from ketoprofen present with concomitant reactions to the perfume ingredient cinnamic alcohol, reactions that at present are difficult to explain by cross-reactivity.21Therefore, patch and photo-patch testing are highly recommended in patients with idiopathic and autoimmune photodermatoses, as well as in all other diseases aggravated by sunlight, in order to detect and avoid exposure to possible aggravating factors, and particularly to UV filters. Recently, recommendations for diagnostic patch testing have been issued by the European Society of Contact Dermatitis (ESCD),22 and in a cooperative effort of the ESCD and European Society of Photodermatology (ESPD), an agreement was not only reached regarding standardized protocols for photo-patch testing,23 but also on the list of 20 allergens to be included in the European baseline photo-patch tests series and an additional extended series including certain classical photo-allergens.24 Last but not least, photo-patch tests with all the patient’s own topical products and systemic photosensitizers to which the patients is exposed are strongly recommended as well, since the outcome may further contribute to the relevance of positive reactions observed, or avoid “false”- negative reactions obtained by testing standardized allergens only.24This number of the Revista da Sociedade Portuguesa de Dermatologia e Venereologia contains two articles dedicated to idiopathic photodermatoses, for which autoimmune reactions to an unknown endogenous chromophore are suspected to be involved – polymorphous light eruption, actinic prurigo, hydroa vacciniforme, chronic actinic dermatitis, and solar urticarial.1,2 Many of these and other photodermatoses have a very clear clinical presentation, while others may mimic allergic contact dermatitis (ACD) or photo-allergic contact dermatitis (PhACD), a classical T cell-mediated or delayed type IV hypersensitivity reaction to an exogenous chromophore applied on the skin in the presence of, or followed by exposure to ultraviolet (UV) or visible light.3,4 Allergic contact reactions can be followed by persistent photosensitivity and chronic actinic dermatitis, such as in cases of chronic ACD from certain plants, e.g., Compositae that are rich in sesquiterpene lactones,5 fragrances, lichens, and colophony,4 or in PhACD or photo-aggravated ACD from drugs like ketoprofen, etofenamate, and chlorproethazine, or even other contact allergens, such as tosylamide/formaldehyde resin, fragrances, and thiourea derivatives.4The long persistence of these chemicals in the epidermis (for up to at least 17 days in the case of ketoprofen),6 or the formation of endogenous photosensitizers might perhaps explain the progression to chronic actinic dermatitis.4In patients with idiopathic photodermatoses the use of sunscreens is mandatory, however, the sensitization risk from these chemicals may be enhanced by the previous skin inflammation and the need for repeated application for long periods.7 UV filters, which are chromophores that capture UV light, are among the most frequent causes of PhACD,8-11 namely benzophenones, dibenzoylmethane derivatives, octocrylene, and cinammates.9,10,12-14 Although more recent UV filters seem to be more photostable and less prone to induce PhACD,3 a few cases have been described,9 for example, from polysilicone-15 (Parsol®SLX).15 With regard to methylene bis-benzotriazolyl tetramethylbutylphenol (syn. bisoctrizole or Tinosorb® M), ACD from it is due to the surfactant decyl glucoside, in particular, which is added in order to stabilize the sunscreen molecule.16,17Topical drugs, such as the non-steroidal anti-inflammatory ketoprofen, piketoprofen, suprofen, etofenamate, piroxicam, and benzydamine,18 as well as phenothiazine derivatives, i.e., promethazine or chlorproethazine, and isothipendyl chlorhydrate19 are frequent causes of ACD/PhACD, either by direct application or by transfer from other individuals in close contact (consort or connubial dermatitis). Moreover, some of these chemicals, particularly ketoprofen, exhibit cross-reactions with UV filters, i.e., benzophenone(s) and octocrylene, the latter containing benzophenone residues. Also fenofibrate, a systemic drug, shares the benzophenone ring and can cross react with ketoprofen and related molecules.3,20 Furthermore, patients with PhACD from ketoprofen present with concomitant reactions to the perfume ingredient cinnamic alcohol, reactions that at present are difficult to explain by cross-reactivity.21Therefore, patch and photo-patch testing are highly recommended in patients with idiopathic and autoimmune photodermatoses, as well as in all other diseases aggravated by sunlight, in order to detect and avoid exposure to possible aggravating factors, and particularly to UV filters. Recently, recommendations for diagnostic patch testing have been issued by the European Society of Contact Dermatitis (ESCD),22 and in a cooperative effort of the ESCD and European Society of Photodermatology (ESPD), an agreement was not only reached regarding standardized protocols for photo-patch testing,23 but also on the list of 20 allergens to be included in the European baseline photo-patch tests series and an additional extended series including certain classical photo-allergens.24 Last but not least, photo-patch tests with all the patient’s own topical products and systemic photosensitizers to which the patients is exposed are strongly recommended as well, since the outcome may further contribute to the relevance of positive reactions observed, or avoid “false”- negative reactions obtained by testing standardized allergens only.2
Fotodermatoses Autoimunes Parte II - Manifestações Clínicas e Terapêutica
Get PDFThe autoimmune photodermatoses are a group of heterogeneous idiopathic dermatoses including five different clinical entities some potentially severe with great impact in patients’ quality of life. General photoprotection measures are sufficient for the treatment of patients with mild forms of disease. In severe disease the therapeutic options are still limited, reflecting the ill-defined physiopathological mechanisms. However recent advances in photoprotection field are contributing to a higher therapeutic success. In an era where photodermatology has been losing its importance, we propose to revise the clinical manifestations and therapeutic options of autoimmune photodermatoses. These are rare and challenging disorders concerning diagnostic and treatment that need a specialized approach by dermatologists.As fotodermatoses autoimunes constituem um grupo heterogéneo de dermatoses idiopáticas incluindo cinco entidades clinicamente distintas: erupção polimorfa à luz, prurigo actínico, hydroa vacciniforme, dermite actínica crónica e urticária solar. Algumas destas dermatoses são potencialmente graves e com grande impacto na qualidade de vida dos doentes. Várias medidas de fotoprotecção são suficientes para o tratamento de doentes com formas ligeiras. As opções terapêuticas actuais são limitadas em doentes com doença grave e refractária, reflectindo os mecanismos fisiopatológicos pouco clarificados. Ainda assim, avanços recentes na área da fotoprotecção têm contribuído para um maior sucesso terapêutico. Numa altura em que a área da fotodermatologia tem vindo a perder gradualmente o seu impacto, propomos rever as manifestações clínicas e abordagens terapêuticas actuais das fotodermatoses idiopáticas, entidades raras e que impõem desafios relevantes no seu diagnóstico e tratamento
Fotoquimioterapia no Eritema Anular Centrifugo Recalcitrante: Uma Opção Terapêutica Promissora?
Get PDFErythema annulare centrifugum is a dermatosis of unknown aetiology which usually follows a self-limiting course. Nevertheless, some cases tend to chronicity, especially when a culprit stimulus is not found, and can be challenging to treat. A 24-year-old male presented with a 3-year history of persistent plaques with polycyclic outlines and an infiltrated rim on the forearms, buttocks and thighs. After histopathological correlation, a diagnosis of erythema annulare centrifugum was therein made. Secondary causes were ruled out. Treatment with topical and systemic steroids provided no benefit, and the patient was reluctant to further systemic oral immunosuppression. Photochemotherapy was then attempted. A marked improved was observed since the first session, and the patient was clear by the 7th treatment. No adverse events were noted. The patient is still in remission at the 3rd month of follow-up. To our knowledge, we report the first case of an erythema annulare centrifugum successfully treated with photochemotherapy. Our case highlights the potential of this time-honoured therapeutic modality to address chronic and debilitating cases of this figurate dermatosis.O eritema anular centrífugo. é uma dermatose de etiologia desconhecida e geralmente auto-limitada. Apesar disto, alguns casos tendem para a cronicidade, especialmente quando não é identificado o estímulo causal. Estes últimos casos são geralmente desafiantes do ponto de vista terapêutico. Um homem de 24 anos recorreu à consulta de Dermatologia por placas policíclicas múltiplas, com crescimento radial, bordo infiltrado e clareamento central, localizadas nos glúteos, antebraços e coxas com 3 anos de evolução. A correlação clinico-patológica conduziu ao diagnóstico de eritema anular centrifugo. Foram excluídas causas secundárias. O tratamento com corticoides tópicos e sistémicos não ofereceu qualquer melhoria, e o doente estava relutante em tentar imunossupressores sistémicos orais. Optou-se assim pela fotoquimioterapia. Uma melhoria muito significativa foi verificada desde o primeiro tratamento, ficando o doente completamente limpo ao sétimo tratamento. Aos 3 meses de follow-up, mantem-se em remissão. No nosso conhecimento, relatamos o primeiro caso de eritema anular centrífugo tratado de forma bem-sucedida com fotoquimioterapia. Enfatizamos o papel que esta modalidade terapêutica clássica ainda pode, e deve ocupar no tratamento de dermatoses inflamatórias refratárias, como o eritema anular centrífugo
Hiperpigmentação da Face Induzida por Tratamento com Olmesartan Medoxomilo-Hidroclorotiazida
Get PDFPhotodistributed hyperpigmentation has been associated with several drugs. We describe a 69-year-old woman who developed facial skin hyperpigmentation starting after treatment with a combination of olmesartan medoxomil and hydrochlorothiazide and improving following its withdrawal, suggesting drug-induced dyspigmentation. Olmesartanmedoxomil-hydrochlorothiazide should be added to the list of drugs that can induce photodistributed cutaneous hyperpigmentation.A hiperpigmentação cutânea com fotodistribuição pode ser despoletada por vários medicamentos. Descrevemos o caso de uma mulher de 69 anos que desenvolveu hiperpigmentação facial após tratamento com olmesartan medoxomilo- hidroclorotiazida e melhorou após a sua suspensão, sugerindo fortemente que a hiperpigmentação foi induzida pelo fármaco. O olmesartan medoxomilo-hidroclorotiazida deve ser adicionado à lista de medicamentos que podem induzir hiperpigmentação cutânea com fotodistribuição
Epidemiologia das infeções fúngicas superficiais em Portugal - revisão de 3 anos (2014-2016)
Get PDFIntroduction: Superficial fungal infections are the most frequent infectious dermatoses and their incidence continues to increase. Dermatophytes are the principal agents presenting, however, a variable geographic distribution.Material and Methods: This study aimed to characterize the epidemiology of superficial fungal infections diagnosed in Dermatology departments/ units of the Portuguese National Health System between January 2014 and December 2016, through a retrospective analysis of the results of positive cultures performed during this period.Results: A total of 2375 isolates from 2319 patients were studied. The most frequently isolated dermatophyte was Trichophyton rubrum (53.6%), which was also the main cause of glabrous skin tinea (52.4%) and of onychomycosis (51.1%). In relation to tinea capitis, Microsporum audouinii was the most prevalent agent globally (42.6%), followed by Trichophyton soudanense (22.1%). While in the Lisbon metropolitan area these dermatophytes were the main causative agents, in the North and Center regions of Portugal, Microsporum canis was the most frequent agent (58.5%). Yeasts were the main agents isolated from onychomycosis of the hands (76.7%).Conclusion: The results of this study are globally in agreement with the scientific literature. Trichophyton rubrum is the most frequent dermatophyte overall. As for tinea capitis, in the Lisbon metropolitan area, the imported anthropophilic species assume particular importance.Introdução: As infeções fúngicas superficiais são as dermatoses infeciosas mais frequentes e a sua incidência continua a aumentar. Os dermatófitos são os principais agentes causais apresentando, contudo, uma distribuição geográfica variável.Material e Métodos: O presente estudo teve como objetivo a caracterização epidemiológica das infeções fúngicas superficiais diagnosticadas nos Serviços/Unidades de Dermatologia pertencentes ao Serviço Nacional de Saúde Português entre janeiro de 2014 e dezembro 2016 através da análise retrospetiva dos resultados das culturas realizadas durante esse período.Resultados: Foram estudados 2375 isolamentos, pertencentes a 2319 doentes. O dermatófito mais frequentemente isolado foi o Trichophyton rubrum (53,6%), tendo sido o principal agente causal da tinha da pele glabra (52,4%) e das onicomicoses (51,1%). Relativamente às tinhas do couro cabeludo, globalmente o Microsporum audouinii foi o agente mais prevalente (42,6%), seguido do Trichophyton soudanense (22,1%). Enquanto na área metropolitana de Lisboa estes dermatófitos foram os principais agentes de tinha do couro cabeludo, nas regiões Norte e Centro o agente mais frequente foi o Microsporum canis (58,5%). Os fungos leveduriformes foram os principais responsáveis pelas onicomicoses das mãos (76,7%).Conclusão: Os resultados deste estudo estão globalmente concordantes com a literatura científica. O Trichophyton rubrum apresenta-se como o dermatófito mais frequentemente isolado em cultura. Na tinha do couro cabeludo, na área metropolitana de Lisboa, as espécies antropofílicas de importação assumem particular destaque
Pioderma Gangrenoso Orbitário Fatal com Envolvimento do Sistema Nervoso Central
Get PDFPyoderma gangrenosum is a chronic inflammatory disease characterized by the development of a painful deep ulcer with undermined borders. Head and neck are rarely affected regions of the body and also usually associated with a worse prognosis. Corticosteroids are the mainstay of treatment although available options are not specific nor completely effective in pyoderma gangrenosum. We report the case of a 46-year-old patient with an aggressive orbital pyoderma gangrenosum with progressive extension to the central nervous system and insufficient response to treatment, ultimately leading to patient’s death.O pioderma gangrenoso é uma doença inflamatória crónica que se caracteriza pelo desenvolvimento de uma úlcera dolorosa e profunda com bordos mal delimitados. Cabeça e pescoço são regiões anatómicas raramente atingidas e geralmente associadas a um pior prognóstico. A corticoterapia é a base do tratamento desta dermatose, embora as opções disponíveis não sejam específicas nem inteiramente eficazes no pioderma gangrenoso. Relatamos o caso de um doente de 46 anos com pioderma gangrenoso orbitário de caráter agressivo, com extensão progressiva ao sistema nervoso central e resposta insuficiente ao tratamento, que culminou na morte do doente