Repositório RCAAP

A dermatomiosite é uma miopatia inflamatória idiopática, rara, com manifestações cutâneas e sistémicas variadas. Mais de 70% dos doentes apresentam autoanticorpos específicos da doença, que se correlacionam com manifestações clínicas distintas. Além dos anticorpos anti-sintetase, nos últimos anos foram descobertos vários anticorpos associados à dermatomiosite e que parecem ter um papel importante na orientação diagnóstica e prognóstica da doença, incluindo os autoanticorpos contra melanoma differentiation antigen 5 (MDA5), transcriptional intermediary factor 1 (TIF1), nuclear matrix protein 2 (NXP2), small ubiquitin-like modifier activating enzyme (SAE) e os anti-Mi2. O autoanticorpo anti-MDA5 está associado a dermatomiosite amiopática e doença intersticial pulmonar rapidamente progressiva e potencialmente fatal, além de caraterísticas mucocutâneas distintas, como ulceração cutânea. Há uma forte correlação entre a positividade para os autoanticorpos anti-TIF-1γ e malignidade, pelo menos na dermatomiosite do adulto. Na dermatomiosite juvenil, estes autoanticorpos têm sido associados a doença cutânea mais extensa, mas não a malignidade. A positividade para anti-NXP2 está associada a calcinose cutânea e envolvimento muscular severos. Na dermatomiosite com autoanticorpos anti-SAE há frequentemente envolvimento cutâneo e disfagia severos, mas com boa resposta à terapêutica imunossupressora e bom prognóstico. Os anticorpos anti-Mi2 associam- -se igualmente a um bom prognóstico.

The rarity of this entity and the exuberance of these cases of white piedra, associated with the complete documentation (including species identification) motivated this report. Mother and daughter presenting asymptomatic tiny white-yellowish nodules, around 1 to 2 mm in diameter, on the hair shaft, for about 6 months. The hot and humid weather and the habit of using conditioning creams may have been determining factors of the infection. Analysis of the hair strands under an optical microscope, culture and microculture and mass spectrometry (MALDI-TOF/MS) concluded the diagnosis of white piedra by Trichosporon ovoides. It resolved with a short haircut associated with ketoconazole 2% shampoo.

Malignant tumours of the lower lip are common and wedge resections are frequently used to address these lesions. Since the lips are paramount for symmetry and overall aesthetics, even minor deformities can lead to disastrous changes. One of these could result from improper intra-operatory antero-posterior alignment between the anterior (cutaneous) and posterior (mucosal) vertex of the wedge, as the latter is not easily determined with precision by the naked eye. The authors present their surgical pearl to accurately define the vertex of the wedge in the posterior surface of the lip. A controlled, antero-posterior transmural penetration at a 90º angle is performed with a hypodermic needle. The entry point is the vertex of the cutaneous wedge. The sharp tip of the needle will now be the reference to the vertex of the mucosal wedge. This and others, easy-to-perform, although critical and frequently overlooked surgical pearls can help the dermatologic surgeon to avoid unwarranted sequelae on a cosmetic sensitive area such as the lips.

Video recording surgical procedures is one of the most valuable tools for teaching and self-assessment in Dermatologic Surgery. A number of recording systems have been described in the literature. Nevertheless, they’re usually expensive (~500€) and time-consuming to prepare, the latter being a major disadvantage for a busy surgical speciality such as Dermatology. The authors describe a simple and inexpensive technique for surgical video capture in Dermatologic Surgery. For this setup, the surgeon needs only a smartphone with a camera (which is nowadays ubiquitous), a bike phone holder (10€ to 30€), an adjustable intravenous pole and, optionally, a powerbank. With the patient in position for the surgical intervention (seated or lying on his back on the surgical table, depending on the procedure), the bike phone holder should be attached to the vertically adjustable intravenous pole. The smartphone must be then set on the bike phone holder, with its “recording mode” already started. Optionally, a powerbank can also be attached to the intravenous pole to provide extra charge for longer procedures. The height of the intravenous pole, as well as its position and distance to the surgical table, should be adjusted accordingly before the intervention, in order to provide the best recording definition. The authors describe a simple, easy to setup, inexpensive system to record videos in Dermatologic Surgery.

Os hemangiomas infantis (HI) são os tumores vasculares benignos da infância mais frequentes, com uma incidência aproximada de 4-5%, sendo mais prevalentes no sexo feminino, recém-nascidos prematuros ou de baixo peso e gravidezes gemelares.A maioria apresenta-se como lesões pequenas e inócuas que, pela sua natureza autolimitada, para além de observação periódica, não necessita de tratamento activo.No entanto, cerca de 10-15% dos HI, seja devido ao seu tamanho ou à sua localização (por exemplo HI perioculares, subglóticos, periorais, perineais...) podem trazer consequências funcionais e estéticas importantes, pelo que necessitam de tratamento activo. Estes casos carecem de observação célere pela Dermatologia e uma intervenção precoce, para que não se perca a janela de oportunidade de início de tratamento e, assim, tentar minimizar efeitos adversos a curto e longo prazo, melhorando os cuidados a estas crianças e reduzindo a morbilidade.Desde 2008, após Léauté-Labrèze ter descoberto acidentalmente a boa resposta do HI ao propranolol, que o paradigma do tratamento dos HI se alterou, passando este a ser recomendado como escolha de primeira linha em 2014.O propranolol oral é um fármaco muito eficaz, com taxas de resposta até 98%, com um perfil de segurança superior aos fármacos tradicionalmente usados no tratamento dos HI (corticoesteróides sistémicos, vincristina, interferão). Deve ser iniciado, idealmente, entre as 5 semanas e os 5 meses de vida (reiterando a importância da referenciação precoce dos HI complicados), na dose de 1 mg/kg/dia, devendo ser aumentado até à dose máxima de 3 mg/kg/dia (dose bidiária), por um período de 6 meses. É um fármaco relativamente bem tolerado, sendo raros e rapidamente reversíveis com a suspensão da terapêutica os efeitos adversos graves (hipoglicémia, bradicardia, broncospasmo).O tempo de duração do tratamento e o modo de suspensão (imediata vs desmame) não são consensuais, havendo casos em que há necessidade de prolongar o tempo da sua administração além dos 6 meses, preconizada na maioria dos casos. Estes pontos devem ainda ser definidos de forma clara para ajudar os clínicos na abordagem dos HI complicados.A utilização do propranolol oral veio revolucionar, sem dúvida, o tratamento dos HI. Lesões complexas, com sequelas funcionais e estéticas potencialmente graves, são tratadas de forma rápida, cómoda, segura e em ambulatório, diminuindo de maneira notável e impressionante a morbilidade associada a estes tumores vasculares.Resta, no entanto, saber se os novos betabloqueantes em estudo, mais selectivos, podem manter ou mesmo ultrapassar a eficácia do propranolol e reduzir os efeitos adversos conhecidos.Este número da Revista da SPDVinclui dois estudos de revisão (1,2) muito clarificadores sobre o estado da arte actual no tratamento dos HI, bem como das eventuais novas terapêuticas que poderão surgir nos próximos anos e, assim, nos auxiliar na abordagem desta entidade tão frequente na Dermatologia Pediátrica.

Introduction: Sentinel lymph node biopsy (SLNB) is the standard of care for cutaneous melanoma, including head and neck melanoma. The aim of this study was to analyze and characterize SLNB in a population of head and neck melanoma patients. Methods: A unicentric, retrospective study on patients with cutaneous head and neck melanoma who underwent SLNB in the Department of Head and Neck Surgery at the Portuguese Institute of Oncology (IPO) Lisbon between January 2010 and December 2017 was performed. The location of primary melanoma, the identification of SLN, the number of the excised SLN, its lymphatic basin origin and the presence of infraclinic metastasis were analysed. Results:  Ninety-eight patients were eligible to undergo SLNB during the observation period. The most frequent locations of primary melanoma were the scalp (24.5%) and the auricular and periauricular region (23.5%) and the most frequent variants were the superficial spreading melanoma (40.8%) and nodular melanoma (30.6%). SLNB was successfully executed in 78 patients (79.6%). A mean of 3.8 lymph-nodes per patient were excised and in 16.7% SLN were excised in more than one lymphatic basin. The SLN were identified in parotid region (39.8%), level II (29.5%) and level V (18.2%). SLN metastases were detected in 13 patients (16.7%). Conclusion: Surgical approach of head and neck cutaneous melanoma is particularly complex. The redundancy of lymphatic system, the multiple SLN and SLN basins influence the SLNB success and may contribute to high rates of false-negatives with its prognostic implications. All patients should be carefully monitored.  

Early malignant syphilis, also called nodule ulcerative syphilis, was first described by Bazin in 1859, related to varying degrees of immunodeficiency, especially human acquired immunodeficiency syndrome (AIDS) caused by human immunodeficiency virus (HIV). It is an aggressive and generalized variant of secondary syphilis with prodromes of fever, headache, myalgia and arthralgia, followed by papulopustular and necrotic eruptions. Because of its rarity and pleomorphic clinical presentation, this condition is underdiagnosed, which can sometimes lead to a fatal outcome. We report a case of malignant syphilis in a 40-year-old male patient with AIDS with exuberant atypical cutaneous manifestation and constitutional symptoms, which has become rare due to antiretroviral therapy.

Cutaneous larva migrans (CML) is a dermatozoonosis characterized by a rash of erythematous lesions, usually prominent, papulo-vesiculous, linear or serpiginous, and most often pruritic. It is caused by penetration and intraepidermal migration of three possible species of nematode larvae. The highest rates of transmission occur in tropical coastal regions, where larvae can migrate freely in sandy soils and where hot temperatures and humidity are ideal for their viability. A case of a 51-year-old male patient who developed a pruritic skin rash on the back, flank, iliac fossa and lower limb on the right is reported. The injuries appeared about seven days after the practice of sport and leisure on turf field. After reviewing the scientific literature, we analyzed the epidemiological, parasitological, clinical, laboratory and therapeutic aspects inherent to the topic of cutaneous larva migrans. It is a common dermatological disease in tropical and subtropical countries, being very important its prompt recognition and therapeutic management for rapid cure with low morbidity.

Reiteramos os nossos sinceros agradecimentos aos revisores que colaboraram na revisão e melhoria dos trabalhos do volume nº76 (2018) da Revista da SPDV. Por lapso não foi publicado o agradecimento ao colega Luís Soares de Almeida, que também participou empenhadamente na revisão e melhoria dos trabalhos publicados no volume em epigrafe. Expressamos deste modo as nossas desculpas ao colega, esperando continuar a contar com a sua preciosa colaboração.

Relatamos o caso de um homem de 79 anos com história de lesões vesiculosas e bolhosas pruriginosas localizadas a nível palmar bilateral. Referia aparecimento há cerca de 2 anos, com resposta parcial a dermocorticóides de baixa-média potência e carácter recidivante. Ao exame objetivo apresentava múltiplas lesões vesiculobolhosas tensas com base eritematosa, a maioria em fase erosiva (Fig. 1). A nível plantar referia lesões no passado, que não apresentava no momento da consulta. Não apresentava outras alterações cutâneas ou mucosas. Foi enviada amostra para cultura de fungos que foi negativa. O estudo histopatológico de biópsia cutânea mostrou descolamento bolhoso subepidérmico com extensa reepitilização subjacente associado a infiltrado linfomononucleado perivascular rico em eosinófilos (Fig. 2). A imunofluorescência direta (IFD) de área perilesional mostrou depósitos lineares de C3 e IgG ao nível da junção dermo- epidérmica. Foram detectados anticorpos circulantes para BP180 (122 U/mL, normal >20 U/mL) por ELISA. Foi iniciado tratamento com prednisolona 30 mg/dia em desmame durante 2 semanas associado a dermocorticóide de alta potência com resolução das lesões e sem formação de novas bolhas em dois anos de follow-up.

Angioedema is a transient localized and self-limiting edema of the subcutaneous and submucosal tissue, due to the release of vasoactive mediators. However, different mechanisms are at the origin of the different types of angioedema (acute or chronic, related or not with mast cell mediators and genetic and familial or acquired). Dermatologists should be able to recognize the different forms of angioedema as associated symptoms, treatment and prognostic factors are completely different. This paper reviews the classification of angioedema, diagnostic tests and management.

Introduction: Malignant melanoma is a very aggressive cutaneous neoplasm, with an increasing incidence in the last years. Material and Methods: A retrospective study was conducted through the analysis of patient’s files diagnosed with cutaneous malignant melanoma between 2011 and 2017. Epidemiological, clinical and histopathological characteristics were analyzed and compared with a previous study from this institution (1999 to 2009). Results: Cutaneous malignant melanoma was diagnosed in 163 patients, 59.5% female and 40.5% male, with a mean age of 65.6 years. The most frequent anatomical location in men was the trunk, while in women it was the leg. The most common subtype of cutaneous malignant melanoma was the superficial spreading, followed by the lentigo maligna and the nodular. The majority of the cases were invasive cutaneous malignant melanoma (67.5%) in opposition to cutaneous malignant melanoma in situ (32.5%). The mean Breslow index was 3.01 mm. It is important to note the increase in the patient’s number with cutaneous malignant melanoma, given that in the previous study only 129 cases had been diagnosed. Conclusion: Our data are in agreement with literature, regarding the patient's age group, and the predominant anatomic location and subtype of cutaneous malignant melanoma. There was an increase in the number of cases located in the head and trunk compared to the lower limbs. Despite the efforts to detect cutaneous malignant melanoma, earlier diagnosis could not counterbalance the global risk of cutaneous malignant melanoma, as we still observe many cases, predominantly invasive and thick.

Nowadays, in clinical practice, when attending a child with a pustular eruption and systemic inflammation, it is mandatory to think of an autoinflammatory disease, once infectious causes have been ruled out. Although rare, autoinflammatory disease must be recognized as early as possible, accurately diagnosed (including gene testing), and treated with targeted therapy if available.

Introduction: Topical immunotherapy with diphenylcyclopropenone is a treatment option for patients with refractory or extensive alopecia areata. The aim of this study was to evaluate the clinical efficacy and tolerability of diphenylcyclopropenone therapy, in patients with alopecia areata, and identify possible prognostic factors that predict response to treatment. Methods: We conducted a retrospective study that included all patients diagnosed with alopecia areata and treated with diphenylcyclopropenone at our Department. Results: Twenty one patients were included for analysis (15 females and 6 males). Overall, nine patients (42.9%) had some hair regrowth with diphenylcyclopropenone therapy. Of these, five (55.6%) achieved pigmented terminal hair regrowth but with persistent patches of alopecia. Only one patient achieved > 90% of hair regrowth. Older age at onset, broader extent of alopecia at baseline and presence of nail dystrophy were all negative prognostic factors (p < 0.05). Atopy and thyroid dysfunction were not statistically significant as predictors of poor treatment outcome. Adverse effects were documented in 15 patients, most of them were mild and did not lead to treatment interruption. Conclusion: Diphenylcyclopropenone therapy is a viable treatment option in patients with extensive alopecia areata, although the response is partial in the majority of the cases. Limitations of this study include its retrospective nature and the limited number of patients.

Kaposi sarcoma is a low-grade vascular tumor, associated with human herpesvirus-8 infection. Solid organ transplant recipients have a much higher risk of this malignancy, with estimated incidence rates increased 84 to 500 times. The risk is even higher in some ethnic groups, already with a higher incidence of this disease. We report a case of a 37-year-old African male, a kidney transplant recipient, with a Kaposi sarcoma refractory to different treatment options. The present case puts in evidence the challenges associated with the management of this disease in this high-risk population and highlights the role of a multidisciplinary approach to achieve effective treatment, particularly in advanced and refractory forms.

Glomuvenous malformations usually present as soft bluish-purple dermal or subcutaneous papules, nodules or plaques, and can occur as sporadic or inherited lesions. We present the case of a 41-year-old female, referred for evaluation of disseminated bluish lesions developing since puberty. Histopathology was consistent with the diagnosis of glomuvenous malformations. The history of a sister with similar lesions justified a genetic study of the glomulin gene that revealed a pathogenic mutational variant allowing thediagnosis of familial disseminated cutaneous glomuvenous malformations. Whereas surgical management is often used for symptomatic solitary lesions of glomuvenous malformations, other treatment modalities have been reported for treatment of multiple lesions, with variable results. The patient underwent sclerotherapy with polidocanol and there were significant symptomatic and cosmetic improvements after six sessions, with no adverse effects and no recurrence after 6 months.

Introduction: Phototherapy, particularly psoralen plus ultraviolet A (PUVA) and narrowband ultraviolet B (nbUVB), is a mainstay in the treatment of Mycosis Fungoides (MF). Methods: We analyzed outcomes of MF patients treated for the first time with either PUVA or nbUVB at the Photodermatology Unit, in the Department of Dermatology of Centro Hospitalar Universitário do Porto, from 2007 to 2017. The primary outcome was response to treatment. The secondary outcome was disease relapse free survival. Results: From a total number of 34 patients with MF identified, nine (26.5%) patients were diagnosed at stage IA and 25 (73.5%) patients were diagnosed at stage IB. Of these patients, 30 (88.2%) were treated with PUVA and four (11.8%) with nbUVB. Overall, 24 (80%) patients treated with PUVA and two (50%) patients treated with nbUVB had complete responses (CR). Relapse was observed in 17 (70.8%) PUVA treated patients and two (100%) nbUVB treated patients. Median disease relapse free survival was 12 months for PUVA and 6 months for nbUVB. Conclusion: PUVA is an effective therapy in early MF. Narrowband UVB could be a valid therapeutic alternative for these patients. However, the small size of nbUVB group in our study limits the conclusions about its efficacy.

The global burden of sexually transmitted infections remains high, with significant associated morbidity and mortality. Gonorrhea is the second most notified sexually transmitted infection in Europe, and its incidence has been increasing in the last years. Although traditionally considered a treatable infection, antimicrobial resistance of Neisseria gonorrhoeaeincludes at present also macrolides, tetracyclines, sulfonamides and trimethoprim combinations, quinolones, and even cephalosporins. These high levels of gonococcal resistance to antimicrobials resulting in untreatable infections in the future may become one of the greatest challenges to the prevention and control of sexually transmitted infections, which may be a significant major public health issue. Therefore, the development of novel antimicrobials and/or new dual antimicrobial therapy regimens is urgently needed. In this paper, evolution of antimicrobial resistance of Neisseria gonorrhoeae is reviewed, along with new drugs currently under development for the treatment of this infection.

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We present an extensive and severe case of confluent and reticulated papillomatosis of Gougerot & Carteaud in a previously healthy adolescent male treated with oral Minocycline, 100 mg per day, for 30 days, with complete remission of the lesions and a sustained response after a 6-month follow-up. Recent literature on the subject is revised.