Myositis Multidisciplinary Clinic in a Tertiary Referral Center.

Background: Idiopathic inflammatory myopathies (IIM) are a rare heterogeneous group of diseases characterised by chronic skeletal muscle inflammation, but other organs are also frequently involved. IMM represent a diagnostic challenge and a multidisciplinary approach is important to ensure successful diagnosis and adequate follow-up of these patients. Objective: To describe the general functioning of our multidisciplinary myositis clinic, highlighting the benefits of multidisciplinary team management in patients with confirmed or suspected IIM and to characterise our clinical experience. Methods: Description of the organization of a dedicated multidisciplinary myositis outpatient clinic, supported by IMM specific electronic assessment tools and protocols based on our Portuguese Register - Reuma.pt. In addition, an overview of our activity between 2017 and 2022 is provided. Results: An IIM multidisciplinary care clinic, based on a close collaboration between Rheumatologists, Dermatologists and Physiatrist is detailed in this paper. One hundred and eighty-five patients were assessed in our myositis clinic; 138 (75%) of those were female, with a median age of 58 [45-70] years. At the last appointment, 130 patients had a confirmed IIM diagnosis, and the mean disease duration was 4 [2-6] years. The most frequent diagnosis was dermatomyositis (n = 34, 26.2%), followed by antisynthetase syndrome (n = 27, 20.8%) and clinically amyopathic/paucimyopathic dermatomyositis (n = 18, 13.8%). Twenty-four patients (18.5%) were on monotherapy and 94 (72.3%) were on combination therapy. Conclusion: A multidisciplinary approach is important to ensure the correct diagnosis and follow-up of these patients. A myositis clinic, with a standardised practice at a tertiary hospital level, contributes to a standardization of care and opens research opportunities.

Cutaneous Markers of Cardiovascular Diseases.

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.

As doenças cardiovasculares são um dos desafios médicos mais importantes a nível mundial devido às suas elevadas taxas de morbilidade e mortalidade. Neste artigo, é feita uma revisão das manifestações cutâneas mais importantes que poderão estar presentes em doentes com doenças cardiovasculares. O reconhecimento atempado destas entidades clínicas é fulcral, uma vez que permite um diagnóstico e tratamento precoces, minimizando os efeitos destas doenças a longo prazo e possivelmente melhorando o prognóstico destes doentes.

Reflectance confocal microscopy for the diagnosis of skin infections and infestations.

Reflectance confocal microscopy (RCM) is a noninvasive real-time imaging technique that has been widely used for the diagnosis of skin cancer. More recently, it has been reported as a useful tool for the diagnosis and management of several inflammatory and infectious skin disorders. This article provides an overview of the current available applications of RCM use in cutaneous infections and infestations. PubMed was used to search the following terms in various combinations: reflectance confocal microscopy, skin, hair, nail, infection, parasitosis, mycosis, virus, bacteria. All papers were accordingly reviewed. In most cutaneous infections or infestations, the main alterations are found in the epidermis and upper dermis, where the accuracy of confocal microscopy is nearly similar to that of histopathology. The high resolution of this technique allows the visualization of most skin parasites, fungi, and a few bacteria. Although viruses cannot be identified because of their small size, viral cytopathic effects can be observed on keratinocytes. In addition, RCM can be used to monitor the response to treatment, thereby reducing unnecessary treatments.

Cutaneous Manifestations of Diabetes Mellitus and Prediabetes.

Diabetes is a serious, chronic disease with a rising prevalence worldwide. Its complications are a major cause of morbidity and mortality and contribute substantially to health care costs. In this article the authors review the most common and sensitive skin manifestations that can be present on patients with diabetes and prediabetes. The prompt recognition of these frequently underestimated entities is extremely important as it may trigger not only an adequate metabolic evaluation but also a timely referral and appropriate treatment, minimizing the secondary effects of long-term diabetes and improving the prognosis of diabetic patients.

A diabetes mellitus é uma doença crónica, com uma prevalência crescente a nível mundial. As complicações da diabetes são uma causa major de morbilidade e mortalidade, condicionando custos importantes na área da saúde. Neste artigo é efetuada uma revisão das manifestações cutâneas mais frequentes presentes em doentes com diabetes e pré-diabetes. O reconhecimento atempado destas entidades é fulcral, levando não só a uma avaliação atempada do perfil metabólico como a uma referenciação e tratamento precoces. Desta forma, é possível minimizar os efeitos secundários da diabetes a longo prazo, melhorando significativamente o prognóstico dos doentes.

Successful Treatment with Fusidic Acid in a Patient with Folliculitis Decalvans.

Dear Editor, Folliculitis decalvans (FD) is a rare form of primary neutrophilic cicatricial alopecia. It is a highly distressing disease that affects young and middle-aged adults, with a slight male predominance (1). The most frequent clinical manifestations are follicular pustules and diffuse and perifollicular erythema that heal with centrifugal scarring. Follicular tufting, erosions, and hemorrhagic crusts can also be present, and this alopecia is most often located at the vertex and occipital area. Patients frequently complain about pain, itching, or burning sensations, and the involvement of other body areas is rare (2). The pathogenesis of this disease remains unclear. Staphylococcus aureus and other hair follicle bacteria can often be isolated from the pustules, suggesting the role of a bacterial infection in its etiology. A defect in the host's immune response can also be postulated by reports of familial cases and the appearance of FD in patients with immunity dysfunctions. Other mechanical factors have been suggested, such as structural abnormalities of the follicle or local inflammation (2). Management of this alopecia is difficult and its course is typically chronic and relapsing. The treatment aim is to stop inflammation and further irreversible destruction of hair follicles. Antibiotics remain the first-line therapy, due both to their anti-inflammatory and antimicrobial properties (1). Although topical fusidic acid is widely used as adjuvant treatment, there are few data regarding its oral use. We report a case of folliculitis decalvans successfully treated with oral fusidic acid. Our patient was a 41-year old Cape Verdean woman with a two month history of alopecia with painful, purulent discharge at the vertex of the scalp. The patient was diagnosed with human immunodeficiency virus type 1 (HIV-1) infection 5 years prior and was stable on her regimen of efavirenz, tenofovir, and emtricitabine, with undetectable viral load. She denied application of topical or capillary products. Dermatological examination revealed a patch of cicatricial alopecia with crusts and follicular pustules (Figure 1). Direct microscopic examination and mycological culture showed no fungal element. A diagnosis of folliculitis decalvans was established and the patient was started on oral fusidic acid at a dose of 500 mg three times a day. Betamethasone dipropionate 0.05% and salicylic acid 3% lotion as well as azelaic acid 5% lotion were also applied to the affected area once daily. After two months of treatment, the patient showed clinical improvement, with less erythema and suppuration of the affected scalp. A partial hair regrowth was noted, mainly at the periphery. Subsequently the patient maintained only topical therapy, and no recurrences were observed after 6-months of follow-up. Fusidic acid is useful in the treatment of skin and soft tissue infections, particularly those due to S. aureus, as shown by randomized controlled studies (3). The clinical efficacy of fusidic acid in the treatment of folliculitis decalvans has been reported previously. Bogg was the first to describe this useful effect (4). Sutter also reported good results with fusidic acid used both topically and orally (500 mg three times a day) (5). However, both failed to report the treatment duration or the outcome on discontinuation. Abeck described three patients that responded to a three week oral course of fusidic acid (500 mg three times a day) and to a maintenance treatment with zinc sulfate (4). During the following year, recurrence was observed in only one patient after ending zinc sulfate therapy. Oral antibiotics are frequently used to treat folliculitis decalvans. Tetracyclines and the combination of clindamycin with rifampicin are the most commonly used (2). However, the disease usually progresses when treatment is stopped. Fusidic acid is an anti-staphylococcal drug with few adverse effects. It is highly bioavailable orally, and has a long plasma half-life. Despite years of clinical use in numerous countries, resistance rates remain at low levels to date (6). Since clinical series or cases including ours have shown good results, this drug should not be forgotten when considering treatment options for folliculitis decalvans.

Neonatal Lupus Erythematosus in a newborn with Citrullinemia.

A 24-day-old female newborn presented with multiple annular erythematous plaques with a targetoid shape, on the forehead, neck and upper trunk, that had begun ten days earlier. She had been diagnosed with citrullinemia type 1 in the first week of life and arginine supplementation was initiated. Her personal and family history was otherwise unremarkable. Laboratory tests showed high levels of anti-La/SSB and anti-Ro/SSA in both mother and baby. The patient was diagnosed with Neonatal lupus erythematosus on the basis of the clinical, histopathological and laboratorial findings. Neonatal lupus is a rare immune-mediated disease that results from transplacental transfer of maternal IgG antinuclear antibodies. The disease can affect different organs, mainly the skin and heart. Although the skin lesions are usually characteristic, they pose a diagnostic challenge in the absence of maternal history.