Consensus on the therapeutic management of atopic dermatitis ‒ Brazilian Society of Dermatology: an update on phototherapy and systemic therapy using e-Delphi technique

Abstract This publication is an update of the "Consensus on the therapeutic management of atopic dermatitis - Brazilian Society of Dermatology" published in 2019, considering the novel, targeted-oriented systemic therapies for atopic dermatitis. The initial recommendations of the current consensus for systemic treatment of patients with atopic dermatitis were based on a recent review of scientific published data and a consensus was reached after voting. The Brazilian Society of Dermatology invited 31 experts from all regions of Brazil and 2 international experts on atopic dermatitis who fully contributed to the process. The methods included an e-Delphi study to avoid bias, a literature search and a final consensus meeting. The authors added novel approved drugs in Brazil and the indication for phototherapy and systemic therapy for AD. The therapeutical response to systemic treatment is hereby reported in a suitable form for clinical practice and is also part of this updated manuscript.

Leprosy and Physiotherapy: a necessary approach / Hanseníase e Fisioterapia: uma abordagem necessária

INTRODUCTION: New cases of leprosy occur due to a set of factors associated with the lack of knowledge about the disease, both by health professionals and patients, favoring late diagnosis, the development of physical and social disabilities, stigma and prejudice. OBJECTIVE: To verify the knowledge of students completing the physiotherapy course on leprosy and professional practice in the care of patients with the disease. METHODS: A qualitative exploratory descriptive study was conducted with 68 undergraduate students from physiotherapy courses from public and private universities (AU, UB, UC), in the State of Mato Grosso do Sul. Data were collected through a questionnaire with ten open questions about knowledge, practical action, motivations, interests and teaching-learning process about leprosy. To organize and analyze the data, the collective subject discourse technique was used. RESULTS: It was found that 60% of aU students, 63% of UB and 30.8% of UC have a general conception about the disease. 46.7% of aU students, 77.8% of UB and 80.9% of UC never had contact with leprosy patients. More than half of the students at the three universities said they were unaware of the physiotherapy approaches and practices in leprosy. Almost 100% of ub and UC students stated that the subject was not addressed during the course and therefore did not feel prepared to provide health education and to guide on how to prevent physical deficiencies resulting from leprosy. 73.3% of AU students, 96.3% of UB and 100% of UC recorded negative evaluations, qualifying the course as precarious, insufficient and weak in the leprosy approach. CONCLUSION: It is concluded that leprosy should be included in physiotherapy courses systematically, providing practical care activities, developing skills from prevention to rehabilitation, seeking greater motivation and identification of his work in this area.

INTRODUÇÃO: Novos casos de hanseníase ocorrem devido a um conjunto de fatores associados à falta de conhecimento sobre a doença, tanto pelos profissionais de saúde quanto pelos pacientes, favorecendo o diagnóstico tardio, o desenvolvimento de incapacidades físicas e sociais, o estigma e o preconceito. OBJETIVO: Verificar o conhecimento de estudantes concluintes do curso de fisioterapia sobre hanseníase e a prática profissional no cuidado ao paciente com a doença. METODOLOGIA: Realizou-se um estudo descritivo exploratório qualitativo com 68 estudantes de graduação dos cursos de fisioterapia de universidades públicas e privadas (UA, UB, UC), no Estado de Mato Grosso do Sul. Os dados foram coletados por meio de questionário com dez perguntas abertas sobre conhecimento, ação prática, motivações, interesses e processo de ensino-aprendizagem sobre a hanseníase. Para organizar e analisar os dados, utilizou-se a técnica do Discurso do Sujeito Coletivo. RESULTADOS: Encontrou-se que 60% dos estudantes de UA, 63% de UB e 30,8% de UC têm concepção geral sobre a doença. 46,7% dos estudantes da UA, 77,8% da UB e 80,9% da UC nunca tiveram contato com pacientes com hanseníase. Mais da metade dos estudantes das três universidades disseram não ter conhecimento das abordagens e práticas fisioterápicas em hanseníase. Quase 100% dos estudantes de UB e UC declararam que o assunto não foi abordado durante o curso e, portanto, não se sentiram preparados para fornecer educação em saúde e para orientar em como prevenir deficiências físicas resultantes da hanseníase. 73,3% dos estudantes da UA, 96,3% da UB e 100% da UC registraram avaliações negativas, qualificando o curso como precário, insuficiente e fraco na abordagem da hanseníase. CONCLUSÃO: Conclui-se que a hanseníase deve ser incluída nos cursos de fisioterapia de forma sistemática, proporcionando atividades práticas de cuidado, desenvolvendo habilidades desde a prevenção até a reabilitação, buscando maior motivação e identificação de seu trabalho nessa área.

Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus foliaceus - Brazilian Society of Dermatology

Abstract: Pemphigus are intraepidermal autoimmune bullous dermatoses that occur with lesions on the skin and / or mucous membranes. The most frequent types are pemphigus vulgaris and pemphigus foliaceus (classic and endemic). This consensus aims to present a complete and updated review of the treatment of these two more frequent forms of pemphigus, based on the literature and the personal experience of the authors. In moderate and severe cases of pemphigus vulgaris and foliaceus, systemic corticosteroid therapy (prednisone or prednisolone) is the treatment of choice. Adjuvant drugs, usually immunosuppressive drugs (azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide) may be prescribed as corticosteroid sparers in refractory cases or with contraindications to corticosteroids to minimize side effects. In severe and nonresponsive cases, corticosteroids in the form of intravenous pulse therapy, immunoglobulin and plasmapheresis / immunoadsorption can be administered. Immunobiological drugs, particularly rituximab, appear as a promising alternative. For milder cases, smaller doses of oral corticosteroid, dapsone and topical corticosteroids are options. At the end flowcharts are presented as suggestions for a therapeutic approach for patients with pemphigus vulgaris and pemphigus foliaceus.

Fogo selvagem: endemic pemphigus foliaceus

Abstract: Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate. Brazil has the world's largest number of patients, mainly in the Central-West region, but the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, with occurrence of familial cases. Anti-desmoglein-1 autoantibodies are directed against desmosomal structures, with loss of adhesion of the upper layers of the epidermis, causing superficial blisters. The etiology is multifactorial and includes genetic, immune, and environmental factors, highlighting hematophagous insect bites; drug-related factors are occasionally involved. Flaccid blisters readily rupture to yield erosive-crusty lesions that sometimes resemble seborrheic dermatitis, actinic keratosis, and chronic cutaneous lupus erythematosus. The clinical presentation varies from localized to disseminated lesions. Clinical suspicion should be confirmed with histopathological and immunofluorescence tests, among others. The progression is usually chronic, and therapy varies according to clinical presentation, but generally requires systemic corticosteroid therapy associated with adjuvant immunosuppressive treatment to decrease the adverse effects of corticosteroids. Once the disease is under control, many patients remain stable on low-dose medication, and a significant proportion achieve remission.

Fogo selvagem: endemic pemphigus foliaceus.

Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate. Brazil has the world's largest number of patients, mainly in the Central-West region, but the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, with occurrence of familial cases. Anti-desmoglein-1 autoantibodies are directed against desmosomal structures, with loss of adhesion of the upper layers of the epidermis, causing superficial blisters. The etiology is multifactorial and includes genetic, immune, and environmental factors, highlighting hematophagous insect bites; drug-related factors are occasionally involved. Flaccid blisters readily rupture to yield erosive-crusty lesions that sometimes resemble seborrheic dermatitis, actinic keratosis, and chronic cutaneous lupus erythematosus. The clinical presentation varies from localized to disseminated lesions. Clinical suspicion should be confirmed with histopathological and immunofluorescence tests, among others. The progression is usually chronic, and therapy varies according to clinical presentation, but generally requires systemic corticosteroid therapy associated with adjuvant immunosuppressive treatment to decrease the adverse effects of corticosteroids. Once the disease is under control, many patients remain stable on low-dose medication, and a significant proportion achieve remission.

Do you know this syndrome? Harlequin syndrome

Abstract: Harlequin syndrome is a rare condition in which one half of the face fails to flush and sweat due to damage of the sympathetic fibers on the ipsilateral side. The majority of cases are idiopathic, but may be iatrogenic or caused by space-occupying lesions or brainstem infarction. We report a case of idiopathic harlequin syndrome in a 34-year-old man with a 5-month history of unilateral facial flushing and sweating after exercise. Despite the rarity of this syndrome, dermatologists should be aware of this condition in order to diagnose properly and provide multidisciplinary assistance.

Cutaneous leiomyosarcoma on the face.

Leiomyosarcoma is a rare skin tumor, most common in white men in the fifth to eighth decades of life. Primary tumors are classified in dermal or subcutaneous, that differ by clinical and prognostic features. They may appear on any site of the body, but are rare on the face. A 54-year-old female was admitted with a 5cm exophytic nodular lesion of 8 months duration on the right cheek, site of previous chronic radiodermatitis. Histopathology revealed spindle-shaped cell neoplasia, positive for smooth muscle actin on immunohistochemistry. Cutaneous leiomyosarcomas on the face are rare and may occur in previously irradiated areas. Immunohistochemistry is mandatory for an accurate diagnosis. Its similarity with other tumors may complicate the diagnosis, with delay expansion of the tumor.

Cutaneous leiomyosarcoma on the face

Abstract: Leiomyosarcoma is a rare skin tumor, most common in white men in the fifth to eighth decades of life. Primary tumors are classified in dermal or subcutaneous, that differ by clinical and prognostic features. They may appear on any site of the body, but are rare on the face. A 54-year-old female was admitted with a 5cm exophytic nodular lesion of 8 months duration on the right cheek, site of previous chronic radiodermatitis. Histopathology revealed spindle-shaped cell neoplasia, positive for smooth muscle actin on immunohistochemistry. Cutaneous leiomyosarcomas on the face are rare and may occur in previously irradiated areas. Immunohistochemistry is mandatory for an accurate diagnosis. Its similarity with other tumors may complicate the diagnosis, with delay expansion of the tumor.

Reconstrução de extensa lesão de orelha com retalho em "porta de Saloon" / Reconstruction of extensive lesion in the ear with a "saloon door" flap

O carcinoma basocelular, também conhecido como epitelioma basocelular, é a neoplasia epitelial mais frequente em nosso meio, sendo mais comum em homens. Relata-se o caso de paciente do sexo feminino, de 75 anos, portadora de um carcinoma basocelular nas regiões da concha, anti-hélice e fossa triangular da orelha direita, que foi completamente excisado, com inclusão da cartilagem. A reparação do defeito foi feita por meio de retalho do tipo "porta de saloon", com bom resultado estético e funcional. O retalho auricular posterior é opção versátil para a reconstrução parcial do defeito da orelha.

Basal cell carcinoma, also known as basal cell epithelioma, is the most frequent epithelial neoplasm in the dermatological practice, being more common in men. The authors report the case of a 75-year-old female patient with a basal cell carcinoma in the concha, anti-helix and triangular fossa regions of the right ear. The lesion was completely excised, including the cartilage. The surgical defect was repaired by means of a "saloon door" flap, which yields good aesthetic and functional outcomes. The posterior auricular flap is a versatile option for partial reconstruction of defects in the ear.

Disseminated tungiasis

Abstract: The authors report an unusual case of disseminated tungiasis in a 52-year-old patient living in the city of Campo Grande, state of Mato Grosso do Sul, who had crusted-papular-nodular lesions located in feet, hands, and right thigh. Diagnosis was confirmed by dermoscopy and anatomopathological examination.

Cutaneous metastases in a patient with no previous diagnosis of cancer: diagnostic challenge

Abstract: On rare occasions, skin lesions are the first local of metastatic manifestation of internal malignancies. In case of no previous diagnosis of these tumors, the approach of suspicious skin lesions becomes a challenge, especially in differentiating cutaneous metastases and adnexal primary neoplasms. Currently, besides epidemiologic, dermoscopic and histopathologic aspects, the evaluation also integrates immunohistochemical exams and cell markers such as p40 and p63, highly specific for skin metastases. This article describes the case of cutaneous metastases as the sole obvious sign of breast cancer in a previously asymptomatic woman. The diagnosis was made by the finding of neoplastic cells in the dermis and immunohistochemistry compatible with ductal carcinoma.

Bullous systemic lupus erythematosus in a 10-year-old child

Abstract: Bullous systemic lupus erythematosus (BSLE) is a rare autoimmune subepidermal blistering disease, with few cases described in childhood. It has different clinical-pathological features. We report a case of BSLE in a 10-year-old child with systemic lupus erythematosus, treated with prednisone and hydroxychloroquine. There was complete remission with dapsone, with no recurrence of skin lesions throughout one year of follow-up. We highlight the rarity and early age of occurrence.

Disseminated cryptococcosis with cutaneous involvement in an immunocompetent patient

Abstract Cryptococcosis is a fungal infection of opportunistic behavior that is unusual in immunocompetent patients. We report a rare case of disseminated cryptococcosis with cutaneous involvement in an immunocompetent individual. During hospitalization, Cryptococcus gattii was isolated from skin lesions, lung and spinal fluid. The diagnosis of disseminated cryptococcosis was confirmed and treatment was established. The patient showed improvement. Due to the probable clinical severity of the disease and the possibility that skin lesions may be the first manifestation of this illness, prompt diagnosis must be established and treatment provided.

Henoch-Schönlein purpura with c-ANCA antibody in an adult

Abstract: The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies.

Disseminated cutaneous atypical mycobacteriosis by M. chelonae after sclerotherapy of varicose veins in a immunocompetent patient: a case report.

Atypical mycobacteria are saprophytic organisms not transmitted from person to person, which affect mainly immunosuppressed but also immunocompetent individuals. We present a case of atypical mycobacteriosis after a vascular procedure, with widespread cutaneous lesions associated with polyarthralgia. Mycobacterium chelonae was identified by the polymerase chain reaction (PCR) method. The patient showed improvement after treatment with three antibiotics. Mycobacterium chelonae causes skin lesions after invasive procedures. The clinical form depends on the immune state of the host and on the entry points. The diagnosis is based essentially on culture and the mycobacteria is identified by PCR. We highlight the importance of investigating atypical mycobacteriosis when faced with granulomatous lesions associated with a history of invasive procedures.

Rendu-Osler-Weber syndrome: dermatological approach.

The Rendu-Osler-Weber syndrome is a rare systemic fibrovascular dysplasia, recognized by mucocutaneous telangiectasias, arteriovenous malformations, epistaxis and family history. Recurrent bleeding, hypoxemia, congestive heart failure, portosystemic encephalopathy, and symptoms related to angiodysplasia of the central nervous system may occur. Since the treatment is based on supportive measures, early recognition is of utmost importance. This article reports the case of a 53-year-old male patient who presented telangiectasias on fingers, oral cavity and nasal mucosa for 10 years, with a history of recurrent epistaxis of varying severity since childhood. Mother, sister and daughter have similar lesions.

Melanoma inhibitory activity in Brazilian patients with cutaneous melanoma.

BACKGROUND: Melanoma inhibitory activity is a protein secreted by melanoma cells and has been used as a tumor marker. Increased Melanoma inhibitory activity serum levels are related to metastatic disease or tumor recurrence. Currently there are no studies on Melanoma inhibitory activity and cutaneous melanoma involving Brazilian patients. OBJECTIVE: To evaluate the performance and feasibility of measuring Melanoma inhibitory activity levels in Brazilian patients with cutaneous melanoma. METHODS: Blood was obtained from ten patients with proved metastatic cutaneous melanoma (Group 1), 15 patients resected for cutaneous melanoma without metastasis (Group 2) and 5 healthy donors (Group 3). Melanoma inhibitory activity was measured using a commercially available ELISA kit. RESULTS: There was a statistically significant difference of Melanoma inhibitory activity levels between patients with and without metastasis (p=0.002), and between patients with metastasis and healthy donors (p=0.002). There was no difference between patients without metastasis and healthy donors (p=0.443). CONCLUSION: Melanoma inhibitory activity is a tumor marker for cutaneous melanoma and the Melanoma inhibitory activity-ELISA test can be easily performed. Patients with metastasis have increased Melanoma inhibitory activity serum levels when compared to patients without metastasis and healthy donors.

Disseminated cutaneous atypical mycobacteriosis by M. chelonae after sclerotherapy of varicose veins in a immunocompetent patient: a case report

Atypical mycobacteria are saprophytic organisms not transmitted from person to person, which affect mainly immunosuppressed but also immunocompetent individuals. We present a case of atypical mycobacteriosis after a vascular procedure, with widespread cutaneous lesions associated with polyarthralgia. Mycobacterium chelonae was identified by the polymerase chain reaction (PCR) method. The patient showed improvement after treatment with three antibiotics. Mycobacterium chelonae causes skin lesions after invasive procedures. The clinical form depends on the immune state of the host and on the entry points. The diagnosis is based essentially on culture and the mycobacteria is identified by PCR. We highlight the importance of investigating atypical mycobacteriosis when faced with granulomatous lesions associated with a history of invasive procedures.

Rendu-Osler-Weber syndrome: dermatological approach

The Rendu-Osler-Weber syndrome is a rare systemic fibrovascular dysplasia, recognized by mucocutaneous telangiectasias, arteriovenous malformations, epistaxis and family history. Recurrent bleeding, hypoxemia, congestive heart failure, portosystemic encephalopathy, and symptoms related to angiodysplasia of the central nervous system may occur. Since the treatment is based on supportive measures, early recognition is of utmost importance. This article reports the case of a 53-year-old male patient who presented telangiectasias on fingers, oral cavity and nasal mucosa for 10 years, with a history of recurrent epistaxis of varying severity since childhood. Mother, sister and daughter have similar lesions.

Melanoma inhibitory activity in Brazilian patients with cutaneous melanoma

Abstract BACKGROUND: Melanoma inhibitory activity is a protein secreted by melanoma cells and has been used as a tumor marker. Increased Melanoma inhibitory activity serum levels are related to metastatic disease or tumor recurrence. Currently there are no studies on Melanoma inhibitory activity and cutaneous melanoma involving Brazilian patients. OBJECTIVE: To evaluate the performance and feasibility of measuring Melanoma inhibitory activity levels in Brazilian patients with cutaneous melanoma. METHODS: Blood was obtained from ten patients with proved metastatic cutaneous melanoma (Group 1), 15 patients resected for cutaneous melanoma without metastasis (Group 2) and 5 healthy donors (Group 3). Melanoma inhibitory activity was measured using a commercially available ELISA kit. RESULTS: There was a statistically significant difference of Melanoma inhibitory activity levels between patients with and without metastasis (p=0.002), and between patients with metastasis and healthy donors (p=0.002). There was no difference between patients without metastasis and healthy donors (p=0.443). CONCLUSION: Melanoma inhibitory activity is a tumor marker for cutaneous melanoma and the Melanoma inhibitory activity-ELISA test can be easily performed. Patients with metastasis have increased Melanoma inhibitory activity serum levels when compared to patients without metastasis and healthy donors. .