Repositório RCAAP

Cutaneous squamous cell carcinoma in situ of the eyelid and periorbital skin malignancies is common and its management is demanding. Surgical excision is considered the first-line treatment, but these techniques have limitations. Topical photodynamic therapy is currently approved for the treatment of squamous cell carcinoma in situ in other areas, but the reports of its use in this sensitive anatomic location area are scarce. Herein we report the case of a 61-year-old-man with extensive periocular Bowen disease that was treated successfully with photodynamic therapy. Photodynamic therapy may be an option as neoadjuvant or curative therapy in selected cases, especially in sensitive anatomic locations where surgery might lead to distortion of the structures or where techniques as micrographically controlled surgery are not available.

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Introduction: Chronic urticaria is a complex multifactorial dermatological disease. It is highly prevalent throughout the world and contributes to lower patients’ quality of life. The purpose of this study is to evaluate the impact of chronic urticaria on the quality of life (QoL) and mental status in Portuguese patients. Methods: This observational study included the evaluation of Portuguese patients from the consultation of chronic urticaria in the Coimbra University Hospital Centre, using several questionnaires including: weekly urticaria activity score (UAS7), Urticaria Control Test (UCT), Dermatology Life Quality Index (DLQI), Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL) and the Hospital Anxiety and Depression Scale (HADS). The results of the questionnaires were posteriorly analysed, and the quality of life and mental status assessed in these patients were correlated with sociodemographic parameters and clinical aspects of chronic urticaria. Results: We studied 112 patients, mainly female (80%), with an average age of 46 years, mostly classified with mild urticaria activity, according to UAS7, and the majority (85%) were poorly controlled, regarding the UCT classification. Using both CU-Q2oL and DLQI we obtained mean values of 48.98 (±19.53) and 7.23 (±7.67), respectively, that show a high impact on the QoL. CU also interferes with mental status values in HADS concordant with anxiety (in a total of 45 patients) and depression (in 22 patients). A strong and positive correlation was found between both instruments used to measure quality of life, with pruritus, number of papules, quality of sleep and the embarrassment caused by cutaneous lesions as the mostly affected parameters in our sample. QoL varies significantly with gender, degree of instruction, clinical severity of symptoms and level of disease control. Although not always directly related, clinical activity of the disease remains the most significant factor of patients' QoL. Anxiety and depression show a significantly relationship with quality of life, however it was not found a significant correlation with the urticaria clinical activity. Discussion: Patient-Reported Outcomes (PRO) proved to be a practical and effective tool in daily clinic assessment of Portuguese patients suffering from chronic urticaria. The variation of quality of life according to sociodemographic characteristics is in conformity with literature. Moreover, new results were achieved regarding the level of instruction. The mostly impaired domains in our sample’s QoL are not totally identical to previous studies, which suggests a meaningful effect of the geographic location. This investigation supported the significant influence of clinical activity of the urticaria and psychological well-being in QoL as previously published. Conclusion: In Portuguese patients with chronic urticaria, it is essential to establish an integrated approach that encompasses the clinical control of urticaria, but also the control of the associated psychological symptomatology.

Impaired skin barrier is one of the hallmarks of atopic dermatitis (AD), with abnormalities in the cornified envelope, lipid lamellae, tight junctions and cutaneous microbiome. These findings are also present in nonlesional skin of AD individuals, suggesting that epidermal barrier defects may be the initial step towards the development of AD and eventually other atopic diseases (atopic march). It is currently known that pathophysiology of AD involves an interplay between this dysfunctional skin barrier and a predominantly type 2 skewed innate and adaptive immune responses, which further disrupt the skin barrier through type 2 cytokines. In this setting, there is enhanced penetration of environmental and food allergens through a deficient barrier, leading to an increased susceptibility to sensitization. During the sensitization process, thymic stromal lymphopoietin (TSLP) polarizes skin dendritic cells to a T-helper 2 response, and TSLP seems to be a key cytokine in the sensitization of food allergy, allergic asthma and rhinitis. In this review, the authors describe the current knowledge of the pathophysiology of the epidermal barrier, its disruption in AD and how it may be involved in the development of atopic comorbidities and the role of barrier repair therapy on the prevention of the atopic march progression.  

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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.

Cryptococcosis is a systemic infection caused by Cryptococcus neoformans, an encapsulated opportunistic yeast. It primarily causes significant infections in immunocompromised individuals and the symptoms vary according to the integrity of the immune system. Cutaneous cryptococcosis affects about 20% of patients with disseminated cryptococcosis, but primary cutaneous cryptococcosis (PCC) without systemic infection is rare. A 76-year-old male patient with chronic obstructive pulmonary disease, hypertension and dyslipidemia, presented with a violaceus inflammatory skin plaque with blisters that progressed despite intravenous ceftriaxone for 7 days. Histopathology of an incisional biopsy was compatible with the diagnosis of cutaneous cryptococcosis. There was a complete response to fluconazole 300 mg/day for 3 months. No systemic disease was detected and there was no evidence of immunosuppression. The importance of including cutaneous cryptococcosis in the differential diagnosis of skin lesions in patients without immunosuppression or the use of immunosuppressive therapy is emphasized. The cutaneous manifestations of the infection can be the first indication for a disseminated disease, therefore, its early recognition is essential to obtain a good prognosis.

Introduction: The vulvar area may be affected by many noninfectious conditions with similar clinical appearance, requiring a cutaneous biopsy. Our goal was to characterize the noninfectious vulvar diseases that required a biopsy in a southwestern Europe Central Hospital during a 10-year period. Methods: A retrospective study of all the noninfectious vulvar diseases with histological confirmation diagnosed in our institution was conducted between January 1, 2008 and December 31, 2017. Results: The sample included a total of 323 biopsies from 317 patients, aged between 11 and 98 years (mean age of 54.2 years). A total of 36 vulvar diseases was identified. Neoplastic conditions were the most frequently found, particularly melanotic macules (22.3%). The most frequent malignant tumor was vulvar intraepithelial neoplasia (6.2%) and squamous cell carcinoma (5.6%). The most common dermatosis was lichen sclerosus (12.7%). Conclusion: Neoplasms were the most frequently diagnosed conditions affecting the vulvar area that required a biopsy. Ruling out malignancy was also the main reason to perform a biopsy. This study highlights the variety of noninfectious diseases that may affect the vulva and require a biopsy. Since vulvar diseases may be serious and carry high levels of patient distress a correct understanding of these conditions is crucial.

Introduction: Superficial mycoses are fungal infections caused mainly by dermatophytes, yeasts and non-dermatophyte filamentous fungi, which affect the most superficial layers of the skin and its appendages. They have a high prevalence worldwide.The aim of this study is to evaluate the epidemiology of superficial mycoses, as well as the index of agreement between direct mycological exa- mination and fungal culture. Methods: This is a retrospective study carried out at the Dermatology clinic of a tertiary hospital, during 6 years. For diagnostic elucidation, ma- terial was collected by scraping or curettage, for further analysis by direct mycological examination and culture for fungi. Results: Four hundred thirty nine samples of suspicious lesions of superficial mycoses from 420 patients were included, 268 female (63.8% pa- tients) with a mean age of 45.7 years (3 months to 95 years), with most cases from the nails (43.4%) and glabrous skin (24.1%). In general, the most common fungus found in the culture was Trichophyton rubrum, however, not in all studied skin sites. Direct mycological examination showed a statistically significant association with culture (K=0.955), if cases with contamination on culture were eliminated. Conclusion: Direct mycological examination and culture, as diagnostic methods in Dermatology, provide satisfactory and low-cost results, favo- ring patients and the health system. This study allowed us to describe the epidemiological profile of patients at a reference dermatology center, with relevant data in relation to our objective. Agreement between direct mycological examination and culture showed the reliability of the me- thods.

Introduction: Atopic dermatitis is a chronic, inflammatory and pruritic dermatosis of high prevalence, especially in childhood, constituting a relevant problem for public health. Several factors are associated with an increased risk for atopic dermatitis: genetic, psychological, infectious, food, environmental, among others, and atopic dermatitis may be associated with other manifestations of atopy, such as asthma and rhinitis. This study aimed to determine the epidemiological profile and the prevalence of personal and family history of allergy in children with atopic dermatitis attended at a reference service, comparing it to the existing literature. Methods: Hospital-based, cross-sectional and descriptive study, involving children of both genders under 18 years old with a clinical diagnosis of atopic dermatitis (Hanifin and Rajka criteria) attended at the Pediatric Dermatology Outpatient Clinic of the Hospital Municipal Universitário de Taubaté (H.MUT), SP – Brazil, from October 2018 to April 2019. Epidemiological data and data related to personal and family history of allergies were collected from medical records. Microsoft Excel 2019 was used for data analysis and compilation. Results: Of the 440 consultations carried out in the period, 35 (7.9%) were for atopic dermatitis, 23 females (65.7%), 15 phototype IV (42.9%) with mean age 7.7 years (standard deviation=4.3), with disease duration from 2 months to 14 years (mean 5.3 years – standard devia- tion=4.3). Thirty-one patients (88.6%) manifested the condition up to the fifth year of life. Personal history of allergies was present in 77.1% (27 individuals), with a predominance of allergic rhinitis and in older age groups. Positive family history in 62.9% (22 individuals). Conclusion: Pioneer study in the region; it allowed the determination of the epidemiological profile of patients with atopic dermatitis at the Pediatric Dermatology Clinic of the H.MUT, revealing an early onset and higher prevalence in females and in individuals with high phototypes. In addition, it identified the prevalence of personal and family history of allergy in these patients, data consistent with the literature.

Sensitive skin syndrome (SSS) can be defined by the occurrence of unpleasant sensations such as stinging, burning, pain or itching after the skin is exposed to physical, chemical or thermal stimuli that would not normally cause these symptoms. With an estimated prevalence of up to 50% in women and up to 30% in men, this condition can be diagnosed using questionnaires and skin reactivity tests. The pathophysiology appears to involve a decrease in epidermal barrier function and neurosensory and immunological dysfunction. Treatment includes general measures, such as avoiding trigger factors, reducing the number of cosmetics applied, and using biocompatible moisturizers. Treatments targeting the signaling pathways affected in the disease, such as TRPV1 inhibitors, are under development.

Nos últimos anos vários estudos demonstraram a implicação da microbiota intestinal em várias doenças de mediação imune como a diabetes, a colite ulcerosa e a esclerose múltipla. Existem poucos dados sobre o microbioma folicular e o seu papel na patogénese de doenças que afetam o couro cabeludo, sendo uma área de investigação crescente. Alguns estudos mostram influência da disbiose nestas doenças, podendo a manipulação do microbioma representar uma possível opção terapêutica. Este artigo procura rever o conhecimento atual relativo ao impacto da disbiose nas doenças dermatológicas do couro cabeludo, como dermatite seborreica, psoríase, alopécia areata, alopécia androgenética, líquen plano pilar, alopécia fibrosante frontal e foliculite decalvante. Uma compreensão alargada deste tema poderá sugerir tratamentos adicionais além das terapêuticas convencionais.

Introduction: Coronavirus disease 2019 (COVID-19) predominantly affects the respiratory system, but there have been many reports of skin involvement. Several cutaneous manifestations associated with COVID-19 have been identified, including acral lesions (pernio-like), which we intend to better characterize in this study. Methods: Demographic, clinical and major histopathologic characteristics of acral skin lesions occurring during the acute infection stage of COVID-19 disease were evaluated in this survey conducted in the Dermatology Division of Santa Casa Hospital Complex, Federal University of Health Sciences, Porto Alegre, Brazil. Results: In the present study, we evaluated eight female patients with acute SARS-CoV-2 infection confirmed by PCR with acral skin lesions including pernio-like. The mean age was 43.6 and with no other acute disease, who had a good outcome with need for hospitalization only in 2 cases. Pernio-like lesions were observed under hot weather conditions in patients who denied any previous history of similar cutaneous manifestations. Some acral lesions resembled those of lupus erythematosus and of herpes simplex paronychia, but laboratory tests were negative for those conditions. Unusual nail involvement was present, characterized by half-moon shaped erythematous bands at the distal margin of the lunula. Conclusion: Recognition of these skin lesions is important in that they could be potential markers, aiding in the diagnosis and prognosis of COVID-19 disease.

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Introduction: Methotrexate has been used in the systemic treatment of psoriasis since 1950 and remains a first-line drug. It is important to assess the factors that influence its discontinuation. The aim of the authors was to identify determinants of drug survival of metho- trexate in patients with psoriasis. Methods: A retrospective analysis was performed concerning patients who started psoriasis treatment with methotrexate between January 2010 and January 2020 and were included in the national registry DERMA.PT by the Centro Hospitalar Universitário São João. Results: A total of 146 patients with psoriasis treated with methotrexate alone or in combination with phototherapy were identified. Most were male (55%), with a mean age of 51±13 years and 65% had psoriasis for more than 10 years. Psoriasis vulgaris (49%) and psoriatic arthritis (47%) were the most common forms of psoriasis, with a mean initial PASI of 10.7±5.6. The majority of patients were methotrexate-naïve. In total, 66 (45%) patients discontinued treatment, with a mean survival time of 18.0±15.5 months. The most common reasons for discontinuation were ineffectiveness (32%), poor compliance (18%) and gastrointestinal intolerance (11%). Previous treatment with cyclosporine and the presence of psoriatic arthritis had both statistically significant associations with the discontinuation of methotrexate. In most cases, a switch to anti-TNFα monoclonal antibody was performed. Discussion: In recent years, retrospective studies have been published focusing on the drug survival of methotrexate in psoriasis, ranging from 12 to 21 months. The main reasons for discontinuation were ineffectiveness and adverse effects, namely gastrointestinal. Our study is in line with what has been described but stands out for the high percentage of patients who maintained treatment. The tremendous evolution in the treatment of psoriasis over the 10-year period of the study greatly influenced the results. The main limitation of this analysis results from its re- trospective nature. Conclusion: The results are in agreement with the published survival characteristics of methotrexate and reinforce its importance and role of this drug in the treatment of this disease.

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Realizou-se na Escola Naval o 24º Encontro do SNHM. Houve 22 conferências, distribuídas pelos dois dias do Encontro. Tiveram lugar duas sessões temáticas, uma sobre a História da Cartografia, a outra de Homenagem a Bento de Jesus Caraça, nos 110 anos do seu nascimento. Nesta sessão a senhora Engenheira Guida Lami não pôde estar presente, sendo a sua comunicação lida por Luís Saraiva, que igualmente apresentou um curto power point da Engenheira Lami «Testemunhos fotográficos inéditos de Bento de Jesus Caraça na Serra da Estrela (1936–1940)», estruturado em torno de fotos do seu arquivo pessoal.

Bento de Jesus Caraça apontava como um dos seus objectivos fundamentais «a criação de uma mentalidade livre e de tonalidade científica entre os cidadãos portugueses». A criação da Biblioteca Cosmos insere-se precisamente nesta linha. Havia muito «que ele acalentava a ideia de construir uma biblioteca popular a qual, sem deixar de ser científica, fosse concebida e redigida de modo a permitir ao homem vulgar, ao não especialista, tomar consciência do mundo que o rodeia e principalmente facultar àqueles que não tinham meios económicos suficientes, bons livros a baixos preços» [3, p. 26]. Os Conceitos Fundamentais da Matemática foram publicados nesta colecção, exprimindo a crença de Caraça que a matemática pode ser desfrutada por todos, desde que apresentada de modo conveniente. Ele sempre explicou a ciência de modo a evidenciá-la como um grande capítulo da vida humana e social. Em particular a matemática, tendo uma especificidade própria, não deixa de ter fundamentos que se inserem, como toda a ciência, na vida real.