Repositório RCAAP

Basal cell carcinoma is the most common skin malignancy of the periorbital region. The authors present a clinical case of a large tumor of the medial canthus which excision results in an extensive periorbital defect. Herein, the authors report a “step-by-step” reconstruction by separated aesthetic units using a simple combination of standard flaps, achieving an excellent final result.

Livedoid vasculopathy is an unusual thrombo-occlusive dermatosis that manifests with punctiform erythemato-purpuric lesions and extremely painful ulcers, preferably in the lower third of the legs. We report five patients with livedoid vasculopathy, three men and two women, aged between 24 and 54 years, with no relevant personal background. The diagnosis was confirmed by biopsy in four cases. Study of thrombophilias revealed heterozygosity of the MTHFR gene in two cases. Treatment was diversified and included among others, venothropics, anticoagulants, antithrombotics, immunosuppressants and intravenous immunoglobulin. Only one patient has unsatisfactory control of the disease. Livedoid vasculopathy etiopathogenesis remains uncertain. It may occur in the context of hereditary or acquired thrombophilia, autoimmunity or neoplasms. These changes should be excluded, but most cases remain idiopathic, which contributes to the therapeutic difficulty. The authors intend to emphasize the importance of the Dermatologist in the diagnosis of a probably under-recognized disease that causes significant morbidity.

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Introduction: Cutaneous squamous cell carcinoma (cSCC) is the second most common skin cancer and its incidence has been rising. The objective of our study was to perform a descriptive and analytical analysis of the cutaneous squamous cell carcinoma excised in the Hospital Center Vila Nova de Gaia e Espinho (CHVNGE) over a period of 10 years and establish trends (incidence, survival and mortality).Material and Methods: Information was retrospectively gathered in the CHVNGE, from January 2004 to December 2013, using the regional cancer registry and the histopathological registry of the hospital. The aim of this study was to describe the characteristics and trends of cutaneous squamous cell carcinoma (incidence, association with actinic keratosis or basal cell carcinoma, survival and mortality rates).Results: 485 cutaneous squamous cell carcinoma were surgically removed in a total of 380 patients (56.1% men and 43.9% women). 361 patients presented invasive cutaneous squamous cell carcinoma and 124 in situ cutaneous squamous cell carcinoma. The Dermatology Department removed 70.4% of the cutaneous squamous cell carcinoma, followed by the Plastics (16.4%) and General Surgery Departments (4.7%). Cutaneous squamous cell carcinoma was more prevalent in the age-group ≥75-years in both sexes (p < 0.001).The mean age of invasive cutaneous squamous cell carcinoma was 76.7 years (±11.5 years), women being older than men (79.0 vs 74.0 years, p < 0.001). The face was the most common topographic location (42.1%), in both genders (p < 0.001). We observed a rising incidence in both genders, particularly in the last study period (16.2/100 000 person-year). The 5-year survival rate was 98.7%. The mean age of in situ cutaneous squamous cell carcinoma was lower than invasive disease (75.5 years ± 11.3). A previous basal cell carcinoma occurred in 20.6% and actinic keratosis were diagnosed more frequently in women (p = 0.040). The face was the most common location (30.8%). Incidence rates have risen, particularly in women and age-group ≥ 75-years.Conclusion: Our study reports a rapid increase of the incidence in an ageing Portuguese population and highlights the importance of improving the existing cancer registries in Portugal.

Sungungual keratoacanthoma is a rare and more aggressive variant of keratoacanthoma. The authors present a case of a 49-year-old male with a one year history of a growing exophytic nodule on the nail bed of the left first toe. Physical examination revealed a two centimeters large exofitic nodule with a verrucous hyperkeratotic central area on the nail bed. A plain radiograph showed a cup-shaped lytic defect in the underlying distal phalanx. Histopathologic analysis revealed a large crater-like squamoproliferative lesion, connected to the epidermis and consisting of lobules and nests of glassy epithelium with numerous dyskeratotic cells, very little degree of cytological atypia and low mitotic activity. Lymphovascular, perineural or bone invasion were not found. Immunohistochemistry with p53 and Ki67 showed exclusive focal basal staining. The diagnosis of subungual keratoacanthoma was made. The main differential diagnosis, both clinical and histological, is with squamous cell carcinoma. It is important to consider this entity to avoid unnecessary diagnostic delays and mutilating treatments.

Introduction: Cutaneous melanoma (CM) is the least common skin cancer, but it accounts for the majority of deaths. This study aimed to evaluate the epidemiological profile of deaths due to CM in the State of São Paulo, as well as to evaluate the factors that may determine their occurrence by gender.Methods: Data on CM mortality in the State of São Paulo, Brazil, between 2005 and 2014 were studied. Descriptive analyzes and univariate and multivariate logistic regression were performed to determine the probability of death in terms of gender according to demographic variables. Statistical analysis was based on OR calculation. In all analyzes, 95% confidence intervals were used and alpha = 5% level of significance was adopted.Results: During the study period 4059 deaths due to CM were identified, representing 0.9% of the general mortality due to neoplasia. Of these, 56.7% were male and 92.4% were white. The mean age at death was 63.5 years (SD = 16.3). In the multivariate logistic regression, the absence of partner and the higher level of education showed to be discordant factors between genders.Conclusion: This study evaluating the epidemiological profile of deaths due to CM, identified the predominance of the male gender, Caucasians and individuals with partners and a low level of education. There was a trend towards an increase in the number of deaths in the last 5 years of the study period. In addition, we observed differences in risk factors related to the gender.

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. 

Vemurafenib, a selective inhibitor of the BRAF V600 mutation, is Food and Drug Administration and European Medicines Agency approved for the treatment of stage IV metastatic melanoma alone or in combination. Among the adverse effects, cutaneous toxicity is the most common. Most of these reactions such as maculopapular rash, photosensitivity and hyperkeratotic lesions are manageable, and the majority of patients are able to continue therapy. However, a few cases of life-threatening severe cutaneous adverse reactions have been reported and drug withdrawal is mandatory in these cases. Herein, we report a case of vemurafenib- -induced toxic epidermal necrolysis in a patient receiving therapy for metastatic melanoma. After several hospital complications, our patient survived to the drug-induced reaction and he is in remission for 2 years.

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Sweet's syndrome is a neutrophilic dermatosis whose etiopathogenesis is not yet fully known. Histopathology of cutaneous lesions shows an infiltrate with mature neutrophils diffusely distributed in the papillary dermis. The diagnosis is made through clinical, laboratory and histopathological criteria. Association of Sweet syndrome and HIV infection is scarcely related in the literature, having been reported with the use of abacavir, opportunistic infections and vaccination. The explicit evidence for the association is considering Sweet's syndrome as a manifestation of immune reconstitution syndrome, which occurs in 10% to 45% of HIV-infected individuals at the start of antiretroviral therapy. The present report describes the case of a patient HIV-positive for 4 years, who maintained antiretroviral therapy irregularity and, after regularizing the use of antiretrovirals, developed Sweet syndrome.

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Introduction: Sexually transmitted infections (STIs) are a significant public health problem worldwide. They are more frequent in young men; however the prevalence in older individuals is increasing. The aim of this study was to access the prevalence of STIs diagnosis in men aged 50 years and older. We also aimed to compare the demographic and behavioral characteristics of those diagnosed with STIs and those with non-STI conditions.Methods: We conducted a retrospective study including all men aged 50 years and older who have attended a Sexual Transmitted Diseases (STD) clinic of a central hospital in Lisbon, Portugal, between July 2015 and December 2017.Results: Between July 2015 and December of 2017,  1722 men attended the STD clinic, of which 323 (18.8%) were aged 50 years and older. Out of these 323 men, 137 (41.4%) were diagnosed with a STI, more often anogenital warts (32.9%), genital herpes simplex virus infection (19.7%) and early syphilis (19.0%). The mean age of the patients with a STI was 59.9 years and almost all (90.5%) reported an inconsistent use of a condom (90.5%). The number of men that have sex with men (MSM) was significantly higher in patients with a STI (p = 0,003) than those with a non-STI diagnosis. Additionally, 40 (29.2%) of these STI patients had been previously infected with the human immunodeficiency virus (HIV) and 33 (24.1%) had a past history for syphilis, which were significantly higher than in patients in patients with a non-STI diagnosis (p = 0.015 and p = 0.001, respectively).Conclusion: Existing data on STI has mainly focused younger population with few studies performed in older individuals. Our study reports a large number of men aged 50 years and older that allows a better understanding of this age group. High levels of unsafe sex and previous diagnosis of HIV and syphilis emphasizes the importance of sexual health promotion in older individuals.

Multiple adult xanthogranuloma is a non-Langerhans cell histiocytosis, a rare variant of the juvenile xanthogranuloma. The etiology is unknown, affecting individuals predominantly below 30 years of age, without predilection for gender. Clinically presents itself as multiple, disseminated, yellow-brownish papules or nodules. Diagnostic confirmation is given by histopathology and by immunohistochemistry, which further allow to distinguish it from Langerhans cell histiocytosis. The spontaneous regression is uncommon. We report a classic, rare and clinically exuberant case of multiple adult xanthogranuloma and took the opportunity to perform a brief literature review on the topic.

Pyoderma 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.

Introduction: Acne vulgaris is a chronic inflammatory disease of the pilosebaceous unit, of multifactorial etiology. It is one of the most common dermatological diseases. It affects more than 85% of teenagers, especially of the male gender. Despite being infrequent in adulthood, recent epidemiological data show a growing prevalence, around 40%, mainly in the female gender, with a negative impact on quality of life. Acne in adulthood or late acne is present after the age of 25 and is classified as late onset acne or persistent acne. The persistent type, which is the most common, represents 70% - 80% of all cases, and is characterised by the persistence of the teenage acne, while late onset acne is defined by the onset after the age of 25, with a prevalence of about 20% to 30%.Objective: To review current scientific literature, especially focused on the pathophysiology of late acne, triggering and aggravating factors, as well as particularities of the therapeutic approach.Methodology: The MEDLINE-PubMed database was used to select original and review articles published between 2001 and 2017.Discussion: Acne can be a clinical sign of systemic disease, mainly with endocrinological abnormalities, as polycystic ovary syndrome, adrenal hyperplasia and virilising tumours. It is important to consider these etiologies into account, especially in women and in the presence of other signs of hyperandrogenism. Several studies point to other triggering or aggravating factors, namely genetic factors, stress, smoking, exposure to ultraviolet radiation, obesity, hyperglycaemic diet, drugs, cosmetics and colonisation by resistant strains of Propionibacterium acnes.Conclusion: In most cases, adult acne affects the face, has mild to moderate clinical severity and is associated with normal hormonal levels. The exclusive location on the lower third of the face is most often associated with other signs of hyperandrogenism and endocrinological disease, as well as with a predominance of inflammatory lesions. Late acne is described as potentially refractory to the conventional therapy and is very relapsing. Thus, it is a therapeutic challenge, which requires an individualized approach.

Introduction: Despite the burden of skin disorders and their physical, psychological and social repercussions, the development of Dermatology and the training of technicians in this discipline face serious challenges in Angola, with a negative impact on the access and quality of care and services provided to the population.Methods: A retrospective study of the Dermatology Training and Development Project was carried out. The Project, launched in 2010 with the support of the Ministry of Health and the Angolan College of Dermatology and Venereology (CADV), aims at increasing the number of dermatologists and the access of patients to clinical referral services. Data on the training activities, other gains and limitations of the Project are presented.Results: Up to 2013, the residency in Dermatology was confined to a single Service in Luanda, which trained eight dermatologists from 1975 to 2013. Five new Regional Dermatology services were opened in 2013 in Cabinda, Malange, Huambo, Benguela and Huíla. As a result the total number of residents in Dermatology increased to 26 in 2017.Conclusion: In the context of Angola, the decentralization of clinical training services, the periodic update of the postgraduate training program and the active promotion of research attitudes and activities are key factors for the development of Dermatology and the provision of referral services closer to the populations.

Atopic dermatitis is a chronic inflammatory skin condition characterized by relapsing pruritic, scaly, erythematous papules and plaques frequently associated with superinfection. It is one of the most common skin diseases affecting 10%-20% of children and 1%-3% of adults, and it is a significant cause of morbidity, quality-of-life impairment and health-care costs. Although many patients can be treated satisfactorily with topical medications and phototherapy, a smaller subset requires more aggressive systemic therapies to control skin inflammation, reduce symptoms, prevent flares, and improve quality of life. Besides, severe atopic dermatitis has a profound effect on many aspects of the patient's life, and a combination of topical and systemic treatment is often required to control the disease, after ensuring all treatment failure causes are adequately excluded. Despite the available classical options, effective and safe treatments for patients with moderate-to-severe atopic dermatitis are limited due to toxicity and side effects. This article reviews the use and the scientific evidence of these medications in the treatment of adult moderate to severe atopic dermatitis, as well as several promising targeted therapies currently in development.

Anogenital infection by human papillomavirus (HPV) is the most common sexually transmitted infection (STI). It bears oncogenic potential, causing most cases of cervical, vulvar, vaginal and anal cancer. There is no specific antiviral therapy and the treatment of HPV-associated neoplasms does not prevent transmission. Thus, prevention of infection is particularly important, and the Venereology consultation is a privileged opportunity to identify people who benefit from preventive measures. The most recent nonavalent HPV vaccine provides coverage for types 6, 11, 16, 18, 31, 33, 45, 52 and 58, which together account for 90% of cancers of the cervix and genital warts. The vaccine is recommended by the WHO for women and men up to 26 years old. In most countries, the vaccine is provided freely to ensure universal immunization of female adolescents prior to the commencement of sexual activity. In addition to the target population, the vaccine may benefit other individuals often treated by dermatologists: men who have sex with men, immunocompromised hosts such as organ-transplant and HIV-seropositive patients, and candidates for immunosuppressive treatments. The decision to vaccinate should consider the individual risk of prior exposure to HPV and the potential benefit of vaccination, which is prophylactic and not therapeutic. We review published data on the immunogenicity, safety and efficacy of the HPV vaccine in different clinical contexts, and international recommendations that may guide individual counseling by the dermatologist.

Histoid leprosy, described by Wade in 1963, represents an atypical variant of lepromatous leprosy, with its own clinical and pathological features. In the past, it was considered a marker of drug resistance and relapse. It presents as keloid-like plaque or nodular lesions. Optical microscopy shows few foamy macrophages and numerous alcohol-acid-resistant bacilli and an infiltrate composed predominantly of spindle cells, which can mimic fibrohistiocytic neoplasms. We report a case of lepromatous leprosy with lesions clinically compatible with the histoid variant in order to demonstrate and reinforce the clinical polymorphism of this type of Hansen’s disease and raise awareness for the need of an early diagnosis and treatment, therefore with the interruption of the transmission chain and reduction of the occurrence of irreversible leprosy-related disability.

Sleep disturbance is common in children with atopic dermatitis, with a significant impact in their quality of life and negative effects on behavior and neurocognitive function. However, the pathophysiology of sleep disturbance in patients with atopic dermatitis is not well understood. It was thought to be consequence of pruritus and scratching, but nowadays we know to a consequence of multiple factors. Recent studies have suggested that melatonin could be involved, not only because of its primordial effect on sleep induction, but also its effects on immunomodulation and as anti-oxidant. In this review, the authors summarize the new insights about the sleep disturbances in children with atopic dermatitis, emphasizing the role of melatonin and its possible therapeutic implication.