Repositório RCAAP

The nasolabial flap represents a valid and well known option to reconstruct defects of the lower lip as in the following case. An 82-year-old male patient with a basal cell carcinoma located on the right lower lip skin had a surgical excision under local anesthesia. The large surgical defect was closed with an inferiorly based pedicle nasolabial flap, with no flap necrosis and acceptable functional and aesthetic results.

Hidradenitis suppurativa (HS) is as a chronic, inflammatory and recurrent skin disease of the hair follicle. Prevalence of HS is a matter of debate, with estimate rates as low as 0.00033% and as high as 4.1%. HS prevalence appears significantly higher in women and has a mean age of onset in the early 20s. The primary histopathologic event seems to be a follicular hyperkeratosis with plugging, dilatation and rupture of the hair follicle resulting with subsequent inflammation and formation of abscesses and sinus tracts. The cause of this is likely multifactorial, involving genetic factors, cigarette smoking, mechanical stress, obesity, immune aberrant response and hormonal abnormalities. HS, as a chronic inflammatory dermatosis, is associated with a variety of concomitant and secondary diseases such as metabolic syndrome, inflammatory and rheumatologic diseases, depression and malignancy, and, for this reason HS management often requires a multidisciplinary team. 

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Due to the worldwide importance of this theme, we present a case of American cutaneous leishmaniasis in a 65-year- -old patient with restricted skin involvement, despite its long evolution (> 3 years). It was a difficult case to diagnose due to the atypical clinical presentation (large ulcer) and inconclusive histopathological study in the initial biopsies. The third biopsy with careful observation and the contribution of an immunohistochemical study allowed a correct diagnosis and cure after 30 administrations of N-methylglucamine antimoniate ev.

Aquagenic keratodermais a rare acquired disorder, most prevalent in young female patients, affecting mainly palmar and plantar regions. Clinically, it presents as translucent edematous papules that after a brief immersion in water turn into a wrinkling skin surface, and resolve after drying the affected region. There are associations with several diseases and Cystic Fibrosis is the most related. We report a peculiar clinical case of the disease, with unilateral edematous hypochromic plaque on left heel with good response to treatment with 3% salycilic acid in vaseline. There was no respiratory involvement.

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Introduction: There is an overlap between clinical and pathological features of the entities known as pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) and white fibrous papulosis of the neck (WFPN). Although the term fibroelastolytic papulosis has been used to encompass both entities as spectrum variants of a one and only disease, many authors still differentiate the two.Methods: Cases of fibroelastolytic papulosis confirmed by histopathologic examination were retrospectively reviewed within a time frame of 2.5 years.Results: Five cases of fibroelastolytic papulosis were identified. All patients were females aged between 63 and 78 years, presenting with an asymptomatic eruption of isolated or coalescing white to yellow papules on the neck. Two of the patients also had involvement of other anatomical areas. In all cases histology demonstrated a significantly decreased or absent papillary dermal elastic plexus, a mild superficial perivascular lymphocytic infiltrate and sparse melanophages in the papillary dermis, without interface changes. A decrease in upper reticular dermal elastic fibers was noted in four cases and thickening of dermal collagen bundles in three cases. Dilated superficial dermal vessels were present in 3 cases and mild epidermal atrophy in one case.Conclusion: We find the differentiation of PXE-PDE and WFPN to be somewhat theoretical, often confusing, and based on non-significant subtle histological and clinical differences. In our opinion, we should not continue to use these terms, but favor the common term of fibroelastolytic papulosis.

Introduction: Scabies is a common cutaneous infestation occurring at any age and, in some communities, it represents a major public health problem. In Portugal, there is a lack of studies about this parasitosis. The aim of our study was to evaluate the principal characteristics of the patients with scabies diagnosed in Department of Dermatology in Centro Hospitalar de Leiria.Material and Methods: We performed a retrospective observational study, including patients diagnosed with scabies observed at the Consultation and Urgency of General Dermatology and at the Consultation and Urgency of Pediatric Dermatology of CHL between January 2015 and December 2016.Results: The study included 109 patients (69 children; 40 adults). The mean age of the patients in the pediatric group was 6.95 years (± 5.92) and 47.33 years (± 22.06) in the group of adults. In 2015, we diagnosed 46 cases of scabies and in 2016, 63 cases were diagnosed, which represents an increment of 37%. The majority of patients (74.3%) was not able to identify the source of contagion. The diagnostic delay was similar in both groups (50 days in the pediatric vs 58 days in the adult group), nonetheless the diagnostic concordance was much higher in the pediatric group (70.8% vs 27.6%, p = 0.000).Conclusion: The authors present the first study of patients affected by scabies in Portugal, showing an increasing incidence, yet probably underestimated. Similarly, other studies have shown that the diagnostic delay is still relevant both in adults and children, however, we highlight higher diagnostic concordance in the pediatric group. The knowledge of the local epidemiologic features of scabies permits to design more consistent preventive strategies and social support.

Genital herpes is a sexually transmitted infection with high global prevalence. Atypical clinical presentations of herpetic infection are rare but are becoming increasingly frequent due to the growing number of immunosuppressed patients. Therapy is challenging, with a longer treatment duration being frequently needed and an important resistance rate to the common anti-viral agents. Foscarnet and cidofovir are effective alternatives. In this article we review three atypical cutaneous presentations of herpetic infection, selected by their relevance in clinical practice: hypertrophic lesions, chronic ulcers and herpetic folliculitis.

Cryptococcosis is a disease caused by the fungus Cryptococcus neoformans, commonly found in bird excrement, like pigeons. This organism has the ability to cause disease in humans, which may affect several organs, including the skin. Most commonly it causes disease in individuals with some kind of suppression of the immune system. The case reported is of a female patient of 44-years- -old that had used corticosteroids for many months without prescription and presented a skin lesion on her left arm. Laboratory tests confirmed the diagnosis of primary cutaneous cryptococcosis.

Introduction: Dermoscopy is an in vivo, non-invasive technique that is widely used as a complementary method for the study of pigmented skin lesions and enables the early diagnosis of cutaneous melanoma. The purpose of the present study is to evaluate the diagnostic accuracy of dermoscopy and to extend the dermoscopy-histopatholoy correlations.Methods: Retrospective study using the database of the Dermoscopy and Pigmented Lesions Clinic and the database of the Histology of the Dermatology Department of CHUC. Each melanocytic lesion was evaluated according to multiple dermoscopic and histopathological parameters. The agreement between diagnoses and between the evaluated parameters was determined.Results: The malignant/benign ratio was 1:9.2. The agreement between the dermoscopic and histologic diagnoses was fair. The agreement for melanoma was excellent and dermoscopy showed 92.9% sensitivity and 96.9% specificity for this diagnosis. There was agreement between a reticular pattern and the presence of bridging of rete ridges and cytological atypia; globular pattern and the presence of nests; atypical network and presence of fibrosis and cytological atypia; blotches and pigmented parakeratosis; blue-whitish veil and fibrosis.Conclusion: In spite of some limitations and unexpected findings, many of the results of the present study are concordant with the literature, showing a high sensitivity and specificity of dermoscopy in the diagnosis of melanoma, which results in improvement in early diagnosis without increasing the total number of excisions of pigmented lesions.

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The knowledge of the pathophysiology of tumour progression is crucial to understand the therapeutic targets in order to control the disease. The mechanisms used by the immune system to affect cancer development and progression has been a challenging question in immunology. It is now postulated that immunology plays a dual role in this process: it protects against tumour growth, destroying “aberrant” tumour cells, but may also promote tumour progression by selecting tumour cells that are able to escape the immune response and survive in an immunocompetent host. These findings gave rise to the concept of “cancer immunoediting”, which explains the influence of the immune system on tumour progression. Several observations like immunosuppression as a risk factor for melanoma, the possibility of partial or complete regression of primary tumour and development of vitiligo, have suggested that melanoma is an immunogenic tumour but a successful tumour evolution can occur in the light of the “immunoediting” concept. Immune checkpoints, cytotoxic T lymphocyte antigen (CTLA)-4 and programmed cell death (PD-1), were recognized to have important roles in regulating T cell responses during tumour development and were proven to be effective targets in treating advanced melanoma. This article will briefly review the process of tumour evolution and its interaction with the immune system as well as the mechanism of action of the immune checkpoint inhibitors to understand better the new targeted immunotherapies for advanced melanoma, that will be further discussed.

Juvenile xanthogranuloma (JXG) is a benign self-limiting histiocytosis, currently classified as a disorder derived from dendrocytes and previously classified as non-Langerhans cell histiocytosis. It usually manifests as a solitary and asymptomatic lesion occurring most often in patients younger than one year of age and most cases resolve completely in three to six years, but systemic involvement may occur. The authors report a case of juvenile xanthogranuloma with an uncommon clinical presentation with disseminated lesions confirmed by the dermatoscopic, histopathologic, and immunohistochemical patterns that allowed the exclusion of possible differential diagnosis.

Merkel cell carcinoma is a rare neuroendocrine tumor localized in photo-exposed areas in aged adults. A high risk of local recurrence, early metastatic disease and, frequently, a late diagnosis, are determinant of a poor prognosis. Dermoscopic examination of this lesion, which is characterized by the presence of multiple atypical vessels and “red-milky areas”, might be a very useful tool to suspect the diagnosis rapidly and early, allowing treatment in due time. The authors report the case of a 71-years-old patient presenting with a Merkel cell carcinoma in the right lower limb in which the dermoscopic findings picked up a high grade of suspicion and allowed tumor excision in a clinically useful and treatable time. The authors also describe the most important dermoscopic findings and patternsobserved in this tumor and the differential diagnosis one must consider in these cases.

Introduction: Psoriasis is an immunemediated dermatosis characterized by chronic inflammation, abnormal proliferation and aberrant differentiation of keratinocytes, hyperplastic blood vessels and infiltration of inflammatory cells. Psoriasis is a multifactorial disease and is influenced by genetics and epigenetic modifications that can be triggered by environmental factors. Climate and sun exposure may affect psoriasis prevalence and the ultraviolet radiation is a useful treatment. Our objective was to evaluate the relationship between the month of birth and the risk of developing psoriasis.Methods: The monthly distribution of births of patients with psoriasis (n = 755) was compared to that of a control population, comprised by Portuguese individual born during the same period and from the same region (n = 6 560 032).Results: Birth rate of psoriasis patients decreased in October (OR 0.74; 95% CI 0.55 – 0.99; p 0.041). After Bonferroni correction and after grouping the months by trimesters, no statistical significant differences were found.Conclusion: Our results may be related to the particular meteorological characteristics of Portugal that may lead to stabilization of the vitamin D synthesis during the whole year. The perception of the environmental influence on the development of a specific pathology is very important because it allows the establishment of preventive measures that will reduce disease incidence. 

Introduction: The incidence of skin cancer has been increasing in recent years around the world. Key targets in the fight against skin cancer include alerting populations to primary prevention by promoting knowledge about risk factors and encouraging better sun protection behaviors as well as secondary prevention promoting skin self-examination and screenings. The Portuguese Society of Dermatology and Venereology and the Portuguese Skin Cancer Association, have collaborated with Euromelanoma, a pan-European information and screening initiative for skin cancer since the year 2000.Material and Methods: This cross-sectional study consisted in the evaluation of data from the completed Euromelanoma questionnaires during the Euromelanoma screening day in Continental Portugal and Islands, in public and private institutions from 2010 to 2016.Results: A total of 9862 individuals were included, 63% female and 37% male, with a mean age of 48 years. The number of clinically suspected malignant or pre malignant lesions was relatively low but consistent with other published Euromelanoma studies: 3% basal cell carcinoma, 0.4% squamous cell carcinoma, 1% melanoma, and 6% of actinic keratoses.Conclusion: The Euromelanoma initiative has raised at national and international level a greater awareness of the population on the theme of skin cancer, with promotion of the messages by the media, assuming itself as an important subject of public health, recognized by the political and sanitary authorities.

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.24

Pain is a major clinical symptom in medicine. In the scope of Dermatology, pain occurs most commonly in the surgical setting, but it is also associated with a wide spectrum of chronic medical entities. On one hand, postsurgical pain is major cause of dissatisfaction and other negative postsurgical outcomes, such as pain chronification. On the other hand, chronic pain is a major cause of suffering and impaired quality of life in dermatological patients. Treatment is frequently challenging, further aggravated by the paucity of evidence-based recommendations for proper pain control in the dermatological care. This is the second and last part of a review on the general principles of acute peri-operative and chronic pain management in Dermatology. In this part, we’ll discuss the treatment of acute pain related with specific therapeutic procedures and the proper management of chronic and painful dermatological conditions.

The autoimmune photodermatoses are a group of heterogeneous idiopathic dermatoses physiopathologically characterized by underexplored immune mechanisms, usually involving an immune reaction against an unknown antigen. This group includes five different clinical entities – polymorphous light eruption, actinic prurigo, hydroa vacciniforme, chronic actinic dermatitis, solar urticaria - some potentially severe with great impact in patients’ quality of life. The pathogenesis of the autoimmune photodermatosis seems to be realated with the formation of photoallergens in response to sunlight. The hypersensibility mechanism varies between the different diseases. The diagnosis depends on the recognition of the clinical presentation. Phototests confirm the diagnosis and allow the determination of the responsible radiation and limiar dose of induction. In an era where photodermatology has been losing its importance, we propose to revise the pathogenesis and diagnosis of autoimmune photodermatoses.