Another Step Towards Indexation of the SPDV Journal in 2018

Mais um Passo para a Indexação da Revista da SPDV em 2018After 2 full years of publication with the new editorial board, 2017 was the year of big changes for the SPDV journal, which we hope will allow the indexation of our journal in the Medline.We had regular and timely publications, each number with an editorial related to one of the main articles published in that number, a continuous medical education (CME) article, followed by 6 articles with original data or review articles on the latest news on dermatology, 6-7 clinical cases, a quiz/what’s your diagnosis presentation taking profit of clinical and histological images and letters to the editors. This format will continue in 2018 to have a more regular type of articles in our journal.2017 was the year of internationalization. Apart from regular Brazilian clinical cases that we really appreciate as most bring new aspects from diseases that are seldom seen among us, we had a CME article on pruritus written by our colleague Manuel Pereira from Germany that with Sonja Ständer and the Munster team is coordinating European studies on pruritus and chronic prurigo, and an editorial written in collaboration with An Goossens the well-known teacher in contact and photocontact allergy from Leuven, Belgium. We are still expecting that our residents or specialists who stay a few months abroad in highly specialized centres with scholarships from the SPDV write review articles in collaboration with the doctors they worked with abroad.This year we began the process of identifying the papers published in our Journal with a DOI (digital object identifier), a direct link to the manuscript that includes the number given to our Journal followed by its initials, the volume and number of each journal and the consecutive number attributed by the webpage to each article that is submitted (ex. https://dx.doi.org/10.29021/spdv.76.2.849). Therefore online submission of all the manuscripts is mandatory. But this is an easy process and allows the authors to trace the state of their manuscripts while in review.It is also our intention in 2018 to begin the process of showing the articles online as soon as they are accepted, before their inclusion on the printed and finally composed volume. We hope to have the collaboration of Dr. José Carvalho who is thoroughly taking care of our webpage.  Thanks to the work of the editorial board and the many invited reviewers* and with a more thorough reviewing process we improved the quality of the papers that we received, although many of them were already of really high standard.Happily this year more original and review articles were submitted (33) of which only 2 were rejected (6%). The rejection rate was much higher within the 75 clinical reports received (38% – 51%). Although some articles submitted in 2017 are still under review the overall rejection rate was around 36%.With the final review of Drª Helena Donato there is a good standardization of procedures, including a critical review of the references, MeSH words and inclusion of all the missing details in the manuscripts to fulfil the international standards for publication of scientific papers. The work of our Editor, Gabriela Marques Pinto, who also makes a final review of all the manuscripts and prepares their composition for printing is also an enormous job.It is our intention to renew the editorial board during 2018 to include other colleagues that have given a significant and timely contribution to our journal, but we will keep the contribution of Dr Helena Donato and her knowledge to submit the journal for indexation with the next number of 2018.Still, to keep with international rules of scientific journals, in 2018 we will change the placing of publicity within the printed journal to avoid its inclusion in the middle of scientific papers.With these changes and improvements we are feeling more confident and on a much better position to achieve indexation of the Revista da SPDV at the Medline. With the contribution of this team and all the dermatologists we hope a favourable decision, but this will be just one step forward as we will certainly keep on improving the Journal of Portuguese-speaking dermatologists.

A Dermatologia e a Pandemia COVID-19

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Lúpus Eritematoso: Manifestações Cutâneas e Tratamento

Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic manifestations. Specific CLE - defined by the presence of an interface dermatitis on histopathological evaluation - is divided into several sub-types, namely acute CLE (ACLE), subacute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), as well as other rarer forms such as LE profundus (LEP). Nonspecific skin findings, such as livedo reticularis or purpura are more frequently seen in patients with systemic disease. Diagnosis requires classification of the subtype, through a combination of physical examination, laboratory studies, histology and sometimes direct immunofluorescence, at the same time ensuring to exclude systemic disease. Regarding the treatment of CLE, antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for recalcitrant disease. Patient education on proper sun protection and avoidance of triggers is crucial. This paper reviews the clinical manifestations of CLE, as well as the treatment.Lúpus eritematoso cutâneo engloba um vasto leque de manifestações dermatológicas, que podem ou não acompanhar- se de acometimento sistémico. As lesões cutâneas específicas – definidas histologicamente pela presença de dermatite de interface – são ainda dividas em subtipos, concretamente lúpus agudo, subagudo ou crónico. O subtipo crónico inclui ainda o lúpus discóide, bem como variantes mais raras, como o lúpus profundo. As manifestações cutâneas não específicas, nomeadamente o livedo reticular ou purpura, são mais frequentes nos doentes com acometimento sistémico. A abordagem diagnóstica implica a correcta subclassificação do subtipo, através duma combinação de exame físico, estudo laboratorial, análise histológica e ocasionalmente imunofluorescência directa, sendo imperativo a exclusão de atingimento sistémico. Do ponto de vista terapêutico, os corticosteróides tópicos e antimaláricos permanecem como a base da terapêutica; no entanto, imunossupressores, análogos da talidomida e anticorpos monoclonais são terapias sistémicas disponíveis para o tratamento da doença recalcitrante. A educação do doente acerca de medidas de fotoproteção e evicção de factores despoletantes é fulcral

Dano Cerebral Associado a Urticária Crónica Espontânea Lembrando Síndroma de Kounis

‘Kounis Syndrome’ is an acute coronary artery event due to an artery spasm occurring during immediate hypersensi- tivity reactions or chronic spontaneous urticaria. Recently it has been reported in other systems, including the cerebral vasculature. We present a case series of three patients observed between January 2016 and December 2018 with acute and transient brain injury associated with concomitant exacerbation of chronic spontaneous urticaria, including one patient with multiple recurrences of neurologic symptoms during exacerbations of urticaria. Minor imaging defects were observed in two patients, but there were no apparent vascular risk factors or coagulation abnormalities that might explain neurologic symptoms. Chronic spontaneous urticaria, through activation of mast cells and mediator release, seems capable of inducing cerebral arterial aggression. The authors want to call the attention to this possible association, reinforcing the need to keep urticaria under control to prevent neurological manifestations.A síndrome de Kounis caracteriza-se por um síndrome coronário agudo devido a vasoespasmo arterial que ocorre em reacções alérgicas imediatas ou na urticária crónica espontânea. Recentemente foi reportado a nível da vasculatura cerebral. Apresentamos três pacientes observadas entre Janeiro de 2016 e Dezembro de 2018 com acidente vascular cerebral agudo ou transitório durante exacerbações de urticária crónica espontânea, incluindo uma doente com episódios neurológicos recorrentes coincidentes com as crises de urticária. Foram detectadas alterações isquémicas cerebrais em duas doentes, mas não foram encon- trados factores de risco cardiovascular ou anormalidades da coagulação que explicassem os sintomas neurológicos. A urticária crónica espontânea, através da activação e libertação de mediadores por parte dos mastócitos, poderá induzir dano a nível da vasculatura cerebral. Os autores enfatizam esta possível associação e a necessidade do controlo das crises de urticária de forma a prevenir manifestações de dano vascular

Patch and Photo-Patch Testing are Important in Patients with Idiopathic Photodermatoses

This 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