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Background: Runs of homozygosity (ROHs), continuous homozygous regions across the genome, are often linked to consanguinity, with their size and frequency reflecting shared parental ancestry. Homozygosity mapping (HM) leverages ROHs to identify genes associated with autosomal recessive diseases. Whole-exome sequencing (WES) improves HM by detecting ROHs and disease-causing variants. Methods: To streamline personalized multigene panel creation, using WES and ROHs, we developed a methodology integrating ROHMMCLI and HomozygosityMapper algorithms, and, optionally, Human Phenotype Ontology (HPO) terms, implemented in a Django Web application. Resorting to a dataset of 12,167 WES, we performed the first ROH profiling of the Portuguese population. Clustering models were applied to predict consanguinity from ROH features. Results: These resources were applied for the genetic characterization of two siblings with epilepsy, myoclonus and dystonia, pinpointing the CSTB gene as disease-causing. Using the 2021 Census population distribution, we created a representative sample (3941 WES) and measured genome-wide autozygosity (FROH). Portalegre, Viseu, Bragança, Madeira, and Vila Real districts presented the highest FROH scores. Multidimensional scaling showed that ROH count and sum were key predictors of consanguinity, achieving a test F1-score of 0.96 with additional features. Conclusions: This study contributes with new bioinformatics tools for ROH analysis in a clinical setting, providing unprecedented population-level ROH data for Portugal.

Sweet’s syndrome (or acute febrile dermatosis) is a neutrophilic dermatosis with a characteristic presentation encompassing specific clinical (fever and erythemato-violaceous oedematous papules, plaques and nodules), laboratory (neutrophilia and increased inflammatory markers), and histological (dermal neutrophilic infiltrate without vasculitis) features. Its pathophysiology is poorly understood but there seems to be an auto-inflammatory component related to mutations in inflammasome genes. It has been subdivided into its classic form, malignancy-associated, and drug-induced, according to its aetiology. The condition usually responds rapidly to steroid therapy, but recurrences are common. This report presents an extremely rare case of hydroxychloroquine-induced Sweet’s syndrome, plus a review of the literature, which encompasses 3 previous cases of Sweet’s syndrome induced by hydroxychloroquine and 1 induced by chloroquine. Despite being relatively easy to diagnose, aetiological investigation poses challenges to the clinician, especially in the elderly population, as several confounding factors might be present. Further studies are necessary to shed light on the pathophysiology behind this entity, to further facilitate diagnostic workup and treatment strategies.

Abstract Introduction Several clinical trials have established the efficacy and safety of dupilumab for treating atopic dermatitis (AD). However, literature remains scarce in reporting the long-term effectiveness, safety, and drug survival of dupilumab in real-world settings. This study aimed to describe the latter outcomes of dupilumab in patients with AD. Methods This Portuguese, multicentric, observational, retrospective study included consecutive adult patients with AD who initiated dupilumab between January 2019 and September 2023, with a follow-up period up to 30 months. Drug discontinuation and adverse effects data were used to estimate drug survival. Clinical assessments included the Eczema Area and Severity Index (EASI), pruritus numerical rating scale (NRS), and Dermatology Life Quality Index (DLQI). Results A total of 312 patients were included in the study, with 56.4% being male (median age of 30 years, range 18–83). The 30-month drug survival rate was 82.0%. During the study period, 12.5% of the sample (n = 39 patients) discontinued treatment: 7.3% due to treatment failure, 2.9% due to safety concerns, 1.3% due to complete disease control, 0.6% due to pregnancy, and 0.3% due to lack of compliance. Adverse events not leading to drug discontinuation were noted in 25.6% of the sample (n = 80). Conjunctivitis was the most frequently reported adverse event (17%), followed by facial erythema (9%). At 30 months, the mean EASI decreased significantly from 27.30 ± 11.89 at baseline to 2.92 ± 3.96 (p

Objectives Generalized pustular psoriasis (GPP) is a rare, life-threatening skin inflammatory disorder. This study aimed to describe the disease course, treatment strategies, and healthcare utilization among patients with GPP in Portugal.Methods This multicentric, observational, retrospective study included consecutive adult patients with GPP undergoing a dermatology evaluation in different reporting institutions by experienced dermatologists between 2002 and 2023.Results A total of 59 patients were assessed. Most of the cohort had a previous history of plaque psoriasis (71%) and 83% presented at least one comorbidity. At the initial encounter, 64% of the cohort needed hospitalization. Systemic involvement was common, including fever (37%), and elevated white blood cell count and erythrocyte sedimentation rate/C-reactive protein (49%). Nearly, 73% of patients initiated systemic drugs, and 70% had to discontinue the first treatment. During the study, 98% of patients experienced at least one flare. At the last visit, 3.4% of patients had died, and 71.2% exhibited signs of active disease despite undergoing treatment.Conclusions Our study demonstrates that GPP is a chronic, debilitating condition associated with systemic involvement, frequent flares, and hospitalizations, despite receiving multiple systemic treatments. Improved disease awareness and new treatments are needed to improve patient care and decrease the burden of the disease.

In 2023, a second wave of the global mpox epidemic, which is mainly affecting men who have sex with men (MSM), was observed in some countries. Herein, we benefited from a large viral sequence sampling (76/121; 63%) and vast epidemiological data to characterise the re-emergence and circulation of the Monkeypox virus (MPXV) in Portugal during 2023. We also modelled transmission and forecasted public health scenarios through a compartmental susceptible-exposed-infectious-recovered (SEIR) model. Our results suggest that the 2023 mpox wave in Portugal resulted from limited introduction(s) of MPXV belonging to C.1.1 sublineage, hypothetically from Asia, followed by sustained viral transmission and potential exportation to other countries. We estimated that the contribution of the MSM high sexual activity group to mpox transmission was 120 (95% CrI: 30–3553) times higher than that of the low sexual activity group. However, among the high sexual activity group, vaccinated individuals likely contributed approximately eight times less [0.123 (95% CrI: 0.068–0.208)] than the unvaccinated ones. Vaccination was also linked to potential reduced disease severity, with a Mpox Severity Score of 6.0 in the vaccinated group compared to 7.0 in unvaccinated individuals. Scenario analysis indicated that transmission is highly sensitive to sexual behaviour, projecting that a slight increase in the MSM sub-population with high sexual activity can trigger new mpox waves. This study strongly supports that continued vaccination, targeted awareness among risk groups and routine genomic epidemiology is needed to anticipate and respond to novel MPXV threats (e.g. global dissemination of clade I viruses).

Hydroxychloroquine is a widely prescribed antimalarial drug to treat immune-mediated diseases, with a good safety profile. We present the case of a 37-year-old woman, Fitzpatrick’s phototype IV, who presented to our outpatient Dermatology Department with a 1-year history of symmetrical blue-gray discoloration on the anterior aspect of the legs. The patient had a diagnosis of anti-synthetase syndrome, treated with prednisolone, hydroxychloroquine, rituximab, cyclosporine, and acetylsalicylic acid. The blood work was unremarkable. The skin biopsy revealed hemosiderin and melanin (Perl’s and Fontana-Masson staining) deposits inside dermal histocytes. The diagnosis of antimalarial-induced hyperpigmentation was established. Cutaneous blue-gray discoloration is a common antimalarial skin toxicity, mainly in women with darker skin. Its pathophysiology is unclear, but local trauma, ultraviolet radiation, and the concomitant use of corticosteroids, anticoagulants, and antiplatelet drugs seem to contribute as triggers. This case illustrates that a high level of suspicion and adequate clinicopathologic correlation is necessary to establish a correct diagnosis.

Abstract Cylindromas are rare, benign adnexal neoplasms primarily found on the scalp and face, with a higher incidence in women and typically manifesting in the second or third decade of life. These tumours can present as single or multiple nodules, with the latter form often linked to CYLD gene mutations, particularly in the context of CYLD cutaneous syndrome. Here, we report a case of a 61‐year‐old male presenting with multiple cylindromas of the scalp, prompting genetic testing that revealed a novel pathogenic variant in the CYLD gene.

Dowling-Degos disease (DDD) is an uncommon genodermatosis. The most closely associated disorder is Galli-Galli disease (GGD). Both conditions are considered on the same disease spectrum, with the differentiating factor being the presence of acantholysis in GGD. A 51-year-old female with a 21-year history of pruritic eruption in flexural areas progressing to the trunk and limbs presented to our dermatology consult. Physical examination revealed reddish-to-dark brown hyperkeratotic papules and reticulated confluent macules. Dermoscopy showed irregular star-shaped brown mottled areas and yellow-brown polygonal structures. Histopathology confirmed features consistent with GGD. Genetic screening identified a mutation in the POGLUT1 gene. Treatment with topical clobetasol propionate 0.05% and oral antihistamines decreased pruritus, but the skin eruption persisted. We present a rare case of GGD describing clinical, genetic, dermoscopy, and histopathological features. Clinicopathological correlation and good cooperation between dermatologists and histopathologists are essential to make the correct diagnosis of GGD.

We presents an unique and significant case of Acute Generalized Exanthematous Pustulosis (AGEP) associated with the use of tadalafil, a phosphodiesterase type 5 inhibitor for erectile dysfunction. AGEP, typically linked to antibiotics and antiepileptics, had not been previously reported with tadaladil in the MEDLINE database. The case involved a 40-year-old male who developed an erythematous rash, fever and sterile pustules after starting tadalafil. A skin biopsy confirmed AGEP. Discontinuation of the drug and treatment with prednsiolone cleared the dermatosis. This case emphasizes the need for awareness among healthcare providers regarding this potential adverse reaction, considering the growing use of tadalafil.

Acute porphyrias are a group of rare genetic metabolic disorders, caused by a defect in one of the enzymes involved in the heme biosynthesis, which results in an abnormally high accumulation of toxic intermediates. Acute porphyrias are characterized by potentially life-threatening attacks and, for some patients, by chronic manifestations that negatively impact daily functioning and quality of life. Clinical manifestations include a nonspecific set of gastrointestinal, neuropsychiatric, and/or cutaneous symptoms. Effective diagnostic methods are widely available, but due to their clinical heterogeneity and non-specificity, many years often elapse from symptom onset to diagnosis of acute porphyrias, delaying the treatment and increasing morbidity. Therefore, increased awareness of acute porphyrias among healthcare professionals is paramount to reducing disease burden. Treatment of acute porphyrias is centered on eliminating the potential precipitants, symptomatic treatment, and suppressing the hepatic heme pathway, through the administration of hemin or givosiran. Moreover, properly monitoring patients with acute porphyrias and their relatives is fundamental to preventing acute attacks, hospitalization, and long-term complications. Considering this, a multidisciplinary panel elaborated a consensus paper, aiming to provide guidance for an efficient and timely diagnosis of acute porphyrias, and evidence-based recommendations for treating and monitoring patients and their families in Portugal. To this end, all authors exhaustively reviewed and discussed the current scientific evidence on acute porphyrias available in the literature, between November 2022 and May 2023.

Basal cell carcinomas (BCCs) and squamous cell carcinomas (SCCs) are high-incidence, non-melanoma skin cancers (NMSCs). The success of immune-targeted therapies in advanced NMSCs led us to anticipate that NMSCs harbored significant populations of tumor-infiltrating lymphocytes with potential anti-tumor activity. The main aim of this study was to characterize T cells infiltrating NMSCs. Flow cytometry and immunohistochemistry were used to assess, respectively, the proportions and densities of T cell subpopulations in BCCs (n = 118), SCCs (n = 33), and normal skin (NS, n = 30). CD8+ T cells, CD4+ T cell subsets, namely, Th1, Th2, Th17, Th9, and regulatory T cells (Tregs), CD8+ and CD4+ memory T cells, and γδ T cells were compared between NMSCs and NS samples. Remarkably, both BCCs and SCCs featured a significantly higher Th1/Th2 ratio (~four-fold) and an enrichment for Th17 cells. NMSCs also showed a significant enrichment for IFN-γ-producing CD8+T cells, and a depletion of γδ T cells. Using immunohistochemistry, NMSCs featured denser T cell infiltrates (CD4+, CD8+, and Tregs) than NS. Overall, these data favor a Th1-predominant response in BCCs and SCCs, providing support for immune-based treatments in NMSCs. Th17-mediated inflammation may play a role in the progression of NMSCs and thus become a potential therapeutic target in NMSCs.

Abstract Blast-induced vibration may be harmful to facilities in the vicinity of operating mines, mainly causing structural damage and human discomfort. This study presents an application of multivariate statistics to predict vibration levels regarding their potential to cause structural damage and human discomfort. An extensive seismic monitoring campaign was executed in a large open-pit iron ore mine, near a small village, to gather a dataset for a predictive multivariate analysis. Ten blasting events have produced a dataset of 158 valid measurements. Three classes of vibration peak velocity were adopted from legal standards, which later supported a cluster analysis. Then, it was possible to compare how much these two classification modalities respond to discriminant analysis. The next step was to carry out a principal component analysis (PCA) from the original database, and, comparatively, to plot both the scores concerning the classes derived from the vibration standard and those from the groups obtained from cluster analysis. PCA has considerably explained the data variability, while the three classes from cluster analysis resulted very similar to the corresponding ones from the vibration standards. The results have demonstrated that multivariate statistics may be applied to manage blasting-induced vibration and its deleterious effects with few adjustments and automation.

Cancer patients may experience several dermatological symptoms during treatment. We describe a case of synchronous presentation of hand, foot, and mouth disease (HFMD) and palmoplantar erythrodysesthesia (PPE) in a breast cancer patient. A 36-year-old female breast cancer patient under chemotherapy presented to our consult with a 1-week history of painful palmoplantar lesions associated with the recent onset of fever and dry cough. Her daughter had been diagnosed with HFMD. There was a positive immunoglobulin M (IgM) and IgG serology for coxsackievirus. We performed two skin biopsies; one specimen was consistent with HFMD, and the other one was with PPE. To the best of our knowledge, the synchronous presentation of HFMD and PPE has not yet been reported in the literature to date. PPE and immunosuppression might have been responsible for the atypical presentation and clinical severity of HFMD.

A monkeypox outbreak has been reported in several countries since early May 2022. Human monkeypox (MPX) diagnosis is based on a clinical suspicion supported by typical skin and mucosal lesions, confirmed with molecular testing. We present the results of all MPX confirmed patients presenting to our department until July 15 of 2022, describing the characteristics of the lesions at diagnosis. In total, 47 patients were included, all men and 44.7% (n = 21) were HIV-positive. Skin lesions were noted in all patients. The most commonly affected area was the genital region (63.8%), followed by the anorectal region (46.8%). Extra anogenital mucosal (oral or conjunctival mucosa) involvement was reported in three patients. Typical skin findings included erythematous papules, whitish, umbilicated papules, some with a necrotic center and an elevated whitish border. Most patients had lesions in multiple phases presenting simultaneously. Correct identification of MPX skin and mucosal lesions is crucial to avoid late diagnosis and prevent further spreading, ensuring less worldwide morbidity.

The resurgence of Mpox, predominantly among men who have sex with men (MSM), has prompted an analysis of its clinical manifestations and epidemiological patterns, particularly in individuals living with human immunodeficiency virus (HIV). This retrospective study aims to delineate and compare the clinical presentations and epidemiological characteristics of Mpox among HIV-positive and HIV-negative individuals. A total of 58 confirmed Mpox cases from a dermatology department in Lisbon were analyzed, focusing on mucocutaneous manifestations, systemic symptoms, and laboratory parameters. Our findings indicate no significant difference in disease severity and presentation between HIV-positive and HIV-negative groups, suggesting that HIV status may not be a determinant of Mpox severity, but rather an indicator of increased sexual risk behaviors, a recognized risk factor for Mpox transmission.

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Human monkeypox (MPX) is an endemic zoonotic disease in regions of Africa caused by the monkeypox virus, with recent outbreaks in several non-African countries. In this study, we present two cases of patients with MPX infection complicated by a deep skin infection. Both patients presented to our dermatology clinic with a clinical syndrome characteristic of MPX. The diagnosis was confirmed based on swabs of skin lesions. Both patients later returned to our clinic with erythema, pain, and edema at the site of previous papules and were diagnosed with deep skin bacterial infection. In this study we provide information on what we believe was an underreported MPX infection complication and give some advice on preventing cases of cellulitis in these patients.

Introduction: Psoriasis is a common, chronic, and inflammatory skin disorder with a high personal, social and economic burden and important implications for healthcare systems. The aim of this study was to provide an epidemiological characterization of individuals with psoriasis in Portugal. Material and Methods: A large observational, cross-sectional, nationwide, population-based survey study developed by the Portuguese Psoriasis Group of the Portuguese Society of Dermatology and Venereology (GPP-SPDV). A structured questionnaire was designed and applied by experienced interviewers to a random, representative sample of Portuguese individuals with psoriasis and/or psoriatic arthritis. Patients were considered to have psoriasis if they replied positively to one of the following questions: “Does any physician have ever diagnosed you with psoriasis?” or “Do you have a skin disorder characterized by scaling, reddish skin lesions located in the elbows/knees/scalp?”. Results: A total of 6381 individuals were interviewed, of which 283 met the criteria for psoriasis, corresponding to a prevalence rate of 4.4% (95% CI 3.95 – 4.98). Out of the participants that met psoriasis criteria, 24% had suggestive signs/symptoms but did not have a clinical diagnosis established and were not being monitored by a physician. Although more than 70% of participants had active disease (scaling, erythema, or pruritus) and one third had joint symptoms, only 12% were on systemic treatment. Fifty percent of participants with psoriasis (n = 139) had relevant comorbidities (most frequently depression/anxiety and cardiometabolic diseases). Sixteen percent of participants with psoriasis (n = 46) reported that psoriasis interfered with their daily activities (median impact of 5 in a 0 – 10 scale) and 12% mentioned the disease had an impact in their sexual life (median impact of 5 in a 0 – 10 scale). Conclusion: The results of this study suggest that the prevalence rate of psoriasis is likely to be high in Portugal, and several gaps exist at different levels of healthcare delivery to these patients, from diagnosis to treatment. This study provides important data for the future planning of interventions targeting the improvement of psoriasis care in Portugal.

Atopic dermatitis is a highly prevalent chronic, immune-mediated inflammatory skin disease with a significant burden on patients, families and healthcare systems. This article presents recommendations developed by the Atopic Dermatitis Group of the Portuguese Society of Dermatology and Venereology addressing several clinical questions that arise in the management and care of moderate-to-severe atopic dermatitis with biologic agents and Janus kinase (JAK) inhibitors based on the available evidence. The recommendations were generated after a thorough evaluation of existing guidelines on the treatment of atopic dermatitis, publications concerning new biologics and JAK inhibitors not yet incorporated into existing guidelines, and expert-based recommendations. It also includes considerations on atopic dermatitis severity, indications for initiating biologic agents and JAK inhibitors, parameters to be considered in the treatment choice, in particular treatment goals, and recommendations for the use, screening and monitoring of these therapies.

Abstract Background: Cutaneous leiomyosarcoma is a rare malignant neoplasm with muscular origin, representing 2%-3% of all cutaneous soft tissue sarcomas. Objectives: The aim of this study was to characterize clinicopathological features of patients diagnosed with cutaneous leiomyosarcoma in our center over the last 20-years. Methods: A retrospective study of patients with a histopathological diagnosis of leiomyosarcoma between 1999 and 2018 was conducted. Results: Eleven patients were diagnosed with cutaneous leiomyosarcoma during this period. Most cases occurred in men (n = 7). Age at presentation ranged from 47 to 92 years (mean 64.9 years). Head and neck were the most frequently involved locations (n = 5). Ten leiomyosarcoma were dermal, with one cutaneous metastasis. Immunohistochemical staining was available for 7 patients, demonstrating positivity for smooth muscle actin in all of them. All neoplasms were treated surgically. Mean survival was 32.2-months. Study limitations: This was a retrospective study based on medical and pathological records. Conclusions: Histopathology is essential for the diagnosis of leiomyosarcoma, usually revealing a dermal or subcutaneous lesion composed of intertwined fascicles of smooth muscle fibers. Immunohistochemistry is then used to adequately differentiate leiomyosarcoma from other spindle cell tumors. When dealing with cutaneous leiomyosarcoma, it is advisable to carefully evaluate the depth of subcutaneous extension, since even minimal subcutaneous involvement may be associated with a poorer prognosis.

Background: The COVID-19 pandemic introduced new challenges in several dimensions in healthcare services. Herein, we describe the real-life strategies and therapeutic options adopted by dermatologists regarding their patients with psoriasis being treated with or with an indication for systemic therapy during the first COVID-19 lockdown period in Portugal. Methods: The study involves a web-based survey on the clinical management of systemic therapy for psoriasis during the COVID-19 pandemic administered to Portuguese dermatologists. The survey consisted of 55 questions (4 openended questions; 51 closed-ended questions), grouped into 6 sections. Results: A total of 60 dermatologists voluntarily participated in this survey. Nearly 63% of the participants opted for suspending biologics during the COVID-19 lockdown period and 23.3% increased the time between drug administrations. Eighty percent of the participants agreed that biologics did not change the probability of acquiring COVID-19 and 58.4% believed that these drugs decreased or did not change the severity of the disease. Approximately one-third of the participants opted not to prescribe a biological agent in patients despite clinical indication over the duration of the pandemic. Nearly 25% of the participants opted for suspending traditional immunosuppressant administration. Virtual appointments were an option for 93.3% of the participants. Conclusion: The COVID-19 pandemic has significantly affected the management of patients with psoriasis being treated with or with an indication for systemic therapy. Some of the decisions made during the first lockdown period were contrary to what we know today. These decisions might have had a significant impact on patients’ quality of life and on future therapeutic success. An adequate interpretation and analysis of the available data will be extremely important to an insightful adaptation of the clinical practice in future confinement or restrictive scenarios.

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Neonatal cutaneous alterations are common, usually appearing at birth or during the first few days of life. Most of these conditions are physiological, benign, and transient, arising from a combination of immaturity of the newborn skin with environmental factors. Nonetheless, some of them may eventually be a clue to underlying disorders. Physicians should therefore be aware of these clinical manifestations so that parents can be reassured and, when necessary, complementary investigations can be undertaken.

Abstract Biological therapies, including anti-TNF agents, are important in the treatment of various chronic inflammatory diseases, including psoriasis, rheumatoid arthritis or inflammatory bowel disease. The increased use of these drugs translates into an increasing awareness of its adverse effects, which include malignancy. In this paper, we describe the case of a 28-year-old woman who developed a spitzoid melanocytic tumor after starting infliximab therapy for ulcerative colitis. The evidence for causality between anti-TNF and melanocytic proliferations is still sparse; nonetheless, treatment-associated immunosuppression seems to play a key role in this phenomenon. Therefore, a regular follow-up with a rigorous skin examination is essential in these patients. Noninvasive techniques such as dermoscopy or reflectance confocal microscopy are particularly useful diagnostic tools in these circumstances.

Cardiovascular diseases are one of the most important global medical challenges because of their high rates of morbidity and mortality. In this narrative review, the authors address the most important dermatologic signs that can be present in patients with cardiovascular disease. The early recognition of these underestimated entities is very important as it may lead to an early diagnosis and timely treatment, thus lessening the effects of long-term disease and possibly improving the prognosis.

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The nose is one of the most frequent anatomic locations of skin cancer. The purpose of surgery is not only fully eliminating the tumor but also preserving nose function and aesthetics. Reconstruction of defects involving the nasal dorsum usually presents a substantial challenge. A wide range of reconstructive options have been developed for reconstructing this area, all of which can result in excellent outcomes. The choice of a reconstructive technique is influenced by tumor type, by the defect, by patient individual skin characteristics, and by surgeon preferences. Defects on the nasal dorsum frequently cross to adjacent cosmetic subunits, such as the nasal tip, the sidewall, the medial canthus, and the glabella. These complex defects usually require challenging reconstructive technique and carry a higher risk of local complications. In this article, the main reconstructive techniques for nasal dorsum reconstruction are reviewed.

Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis. It is characterized by a recurrent eruption of vesicles, erosions, and scaly erythematous plaques involving intertriginous areas and first occurring after puberty, mostly in the third or fourth decade. In 2000, mutations in the ATP2C1 gene on band 3q22.1, encoding the secretory pathway Ca2+/Mn2+-ATPase protein 1(hSPCA1), have been identified as the cause of HHD. We report the identification of two novel mutations of ATP2C1 gene in two Portuguese patients, which expands the spectrum of ATP2C1 mutations underlying HHD and provides useful information for genetic counseling.

Schwannoma is a benign encapsulated nerve sheath tumor composed of Schwann cells, that may arise anywhere along the course of a nerve. Cutaneous schwannomas, associated with peripheral nerves, usually present as a slow-growing well-circumscribed nodule, localized in the dermis or subcutaneous tissue. They are generally asymptomatic; however, they may become painful by the nervous compression. Since the clinical signs are non-specific, histology is required for a definitive diagnosis. Herein, we present a case of a solitary cutaneous ancient schwannoma of the ear, a histopathological variant of schwannoma with distinctive morphological characteristics.

Objetivo: apreender as representações sociais elaboradas nos portais jornalísticos sobre a enfermagem durante a pandemia da covid-19. Síntese de conteúdo: estudo documental e descritivo-exploratório fundamentado na teoria das representações sociais. Coletaram-se notícias de portais jornalísticos mais acessados no Brasil vinculados à mídia impressa, publicadas entre 26 de fevereiro e 12 de maio de 2020. Analisaram-se os dados por meio do software Analyses Multidimensionnelles de Textes et de Questionaires, o Iramuteq, versão 0.7 alpha 2. A análise das notícias revelou conteúdos representacionais sobre os profissionais de enfermagem como aqueles que cuidam de pessoas, que precisam de proteção para continuar cuidando e que são a maior força de trabalho nos sistemas de saúde. Conclusão: o fenômeno do novo coronavírus tem permitido a transformação dos conteúdos representacionais e a ressignificação de imagens identitárias sobre quem são os profissionais de enfermagem e sua importância para a saúde.

ABSTRACT: Background: Despite all efforts, the demand for organs increases. New and better strategies are still needed, critical in a crisis like pandemics. Methodology: A mathematical approach that integrates need, Opportunity, and Accessibility to kidney transplantation, was created. NOA method, corresponds to the lateral surface area of a trigonal pyramid with the need, Opportunity, and Accessibility as axis, resulting in an intuitional chart output (NOA chart) and a percentage score (NOA score). Higher NOA scores are associated with larger NOA chart areas. Method Application: We found some natural variability among the European Member States regarding Need, Opportunity, and Accessibility to kidney transplantation, concomitant with NOA scores. In 2019, in the European Union, 129 patients pmp on the waiting list for a kidney transplant were registered, 47 kidneys pmp were procured, and 36 kidneys pmp were transplanted, corresponding to 25% of kidney transplantation's response capacity. Conclusion: Transplantation is frequently the better treatment for end-stage kidney failure. NOA method may be, in the future, an indicator for evaluating the overall transplantation performance regarding the need for it and a tool for policy definition. With NOA method we seek to contribute for: • A transplantation overall performance normalizing score; • Transplantation response capacity evaluation.

Introduction: Several new treatments have been developed for psoriatic disease, an inflammatory condition that involves skin and joints. Notwithstanding, few studies have made direct comparisons between treatments and therefore it is difficult to select the ideal treatment for an individual patient. The aim of this systematic review with network meta-analysis (NMA) was to analyze available and approved biologic therapies for each domain of psoriatic disease: skin, peripheral arthritis, axial arthritis, enthesitis, dactylitis, and nail involvement.Methods: Data from randomized clinical trials (RCTs) were included. A systematic review was performed using the MEDLINE database (July 2020) using PICO criteria. Bayesian NMA was conducted to compare the clinical efficacy of biological therapy in terms of the American College of Rheumatology criteria (ACR, 24 weeks) and Psoriasis Area and Severity Index (PASI, 10–16 weeks).Results: Fifty-four RCTs were included in the systematic review. Due to the design of the RCTs, namely, outcomes and time points, network meta-analysis was performed for skin and peripheral arthritis domains. For the skin domain, 30 studies reporting PASI100 were included. The peripheral arthritis domain was analyzed through ACR70 in 12 studies. From the therapies approved for both domains, secukinumab and ixekizumab were the ones with the highest probability of reaching the proposed outcomes. There is a lack of outcome uniformization in the dactylitis, enthesitis, and nail domains, and therefore, an objective comparison of the studies was not feasible. Nevertheless, secukinumab was the treatment with the best compromise between the number of studies in each domain and the results obtained in the different outcomes.Conclusion: Secukinumab and ixekizumab were the treatments with the highest probability of reaching both PASI100 and ACR70 outcomes. Due to the lack of a standard evaluation of outcomes of the other psoriatic disease domains, a network meta-analysis for all the domains was not possible to perform.

Atualmente, com o desenvolvimento recente de agentes biológicos e outros novos fármacos, alguns dos quais com o seu uso já aprovado em idade pediátrica, torna-se importante contextualizar o papel da fototerapia na dermatologia. Esta tem um perfil de segurança excelente e bem reconhecido desde há décadas, para além de não necessitar de monitorização laboratorial, o que constitui uma mais valia, sobretudo na população pediátrica. Tem sido utilizada com sucesso nesta faixa etária no tratamento de diversas doenças dermatológicas. Neste artigo é realizada uma revisão acerca das principais particularidades da utilização da fototerapia em idade pediátrica, focando os efeitos adversos, os principais obstáculos, as suas indicações e a seleção individualizada das diferentes modalidades, de modo a facilitar a prática clínica desta terapêutica.

Abstract Knowledge of particulate system properties is very important in various industrial instances and the possibility of fast predicting the behavior of such systems is an important control tool. The specific surface area of simulated particulate systems was studied as a function of the sharpness parameter of the size distribution of the Gates–Gaudin-Schumann, Gaudin–Meloy and Rosin–Rammler equations. The results showed good statistical adherence, especially the Rosin–Rammler equation, in situations where it is the best descriptor of particle size distribution.

A dermatoscopia é uma técnica não-invasiva, que permite aumentar a acuidade diagnóstica de diversas lesões cutâneas. Sendo um procedimento indolor, a dermatoscopia é particularmente útil nas crianças, poupando-as de biópsias e terapêuticas desnecessárias. Na parte II deste artigo, destacamos a importância da dermatoscopia para diagnóstico e acompanhamento de dermatoses infeciosas e inflamatórias na idade pediátrica.

As lesões melanocíticas e outros tumores cutâneos constituem um dos principais motivos de consulta de dermatolo- gia pediátrica. Apesar de serem frequentemente benignos, alguns podem simular o melanoma, sendo um motivo de ansiedade para os pais e clínicos. A dermatoscopia é uma técnica não-invasiva e indolor, que auxilia o diagnóstico de tumores cutâneos, poupando os doentes a biópsias desnecessárias. Na parte I deste artigo, destacamos a importância da dermatoscopia para diagnóstico de patologia cutânea tumoral na idade pediátrica.

With an increasing prevalence during the past decades, atopic dermatitis has become a global health issue. A literature search following a targeted approach was undertaken to perform this non-systematic review, which intends to provide an overview of the epidemiology, pathophysiology, clinical features, comorbidities, and current therapies for the treatment of atopic dermatitis. In sum, this is a heterogeneous skin disorder associated with variable morphology, distribution, and disease course. Although not completely understood, its pathogenesis is complex and seems to result from a combination of genetic and environmental factors that induce skin barrier dysfunction, cutaneous and systemic immune dysregulation, skin microbiota dysbiosis, and a strong genetic influence. Diagnosis is based on specific criteria that consider patient and family history and clinical manifestations. Overall disease severity must be determined by evaluating both objective signs and subjective symptoms. Therapeutic goals require a multistep approach, focusing on reducing pruritus and establishing disease control. Patients should be advised on basic skin care and avoidance of triggers. Topical anti-inflammatory agents should be considered in disease flares or chronic/recurrent lesions. In case of inadequate response, phototherapy, systemic immunosuppressants and, more recently, dupilumab, should be added. Nevertheless, the treatment of moderate-to-severe atopic dermatitis remains challenging and novel, efficacious, safe and targeted treatments are urgently needed. In conclusion, although the last few years have seen important improvement in the understanding of the disease, future research in atopic dermatitis will continue exploring gene-environment interactions and how it affects pathophysiology, disease severity, and treatment outcomes.

Introdução: A celulite e a erisipela constituem a causa mais frequente de internamento no Serviço de Dermatologia do Hospital Santa Maria. Este estudo teve como objetivo investigar se as características demográficas, as comorbilidades, a existência de episódios prévios de celulite/erisipela, a presença de complicações associadas, os parâmetros laboratoriais na admissão, o isolamento de microrganismo em cultura ou o uso prévio de antibióticos estão associados a internamentos prolongados. Material e Métodos: Estudo retrospetivo, incluindo os doentes internados no Serviço de Dermatologia do Hospital Santa Maria com o diagnóstico de celulite/erisipela, entre 1 de julho de 2012 e 30 de junho de 2017. Resultados: Existiram 372 internamentos, correspondendo a 348 doentes. A mediana do tempo de internamento foi de 11 dias. A idade (p = 0,002, OR 1,03, 95% IC 1,01 – 1,04), a existência de internamento prévio por celulite/erisipela (p = 0,005, OR 4,81, 95% IC 1,63 – 14,23), a presença de complicações associadas à celulite/erisipela (p = 0,001, OR 3,28, 95% IC 1,63 – 6,59), a leucocitose (p = 0,049, OR 1,81, 95% IC 1,00 – 3,30), valores elevados de proteína C reativa (p = 0,035, OR 1,03, 95% IC 1,00 - 1,06) e o isolamento de microrganismo em cultura (p = 0,002, OR 2,59, 95% IC 1,41 – 4,79) estiveram associados a internamentos prolongados. Discussão: A par dos maiores custos associados, o internamento prolongado por celulite/erisipela está frequentemente associado à necessidade de investigação clínica adicional, a tratamentos invasivos, a cursos prolongados de antibioterapia, ao risco de infeções nosocomiais e ao atraso no retorno às atividades da vida diária. Assim, o estudo dos fatores clínico-laboratoriais associados ao internamento prolongado por celulite/erisipela é fundamental e poderá ser útil para a construção de um score de gravidade. Conclusão: O conhecimento de características clínicas e laboratoriais associadas ao internamento prolongado poderá ser relevante para melhorar os cuidados de saúde, através da redução dos tempos de internamento e dos seus riscos e custos associados.