Abstract The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors’ intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

RESUMO Introdução: A dermatite atópica (DA) é uma doença inflamatória crônica e multifatorial. Casos graves afetam a qualidade de vida. A patogénese é complexa, marcada por resposta imune celular defeituosa, exacerbação da resposta inflamatória Th2 e comprometimento da função de barreira da pele. Evidências sugerem que a vitamina D atua na regulação da resposta imune inata e adaptativa, na redução do processo inflamatório e na melhora da barreira cutânea. O nosso objectivo foi correlacionar os níveis séricos de vitamina D com a gravidade da DA. Material e Métodos: Foram analisados prontuários de 30 pacientes assistidos em um ambulatório de Dermatologia, entre 2015 e 2018. Os pacientes foram divididos em 2 grupos: doença leve (terapia tópica) e doença moderada a grave (usuários de imunossupressores). Resultados: Foram selecionados 10 casos com DA leve e 20 com DA moderada a grave. Entre os pacientes com doença leve, 20% apresentaram deficiência de vitamina D. Quanto aos pacientes com doença moderada a grave, 40% apresentaram vitamina D deficiente. Comparando a média de vitamina D nos dois grupos, não houve diferença estatística entre o valor médio. Conclusão: Estudos sobre associação entre os níveis de vitamina D e gravidade da DA permanecem controversos. Alguns trabalhos reforçam essa associação. Neste estudo, apesar da proporção de pacientes com deficiência de vitamina D ser duas vezes maior entre os pacientes com DA moderada a grave, não houve significância estatística entre os dois grupos, corroborando estudos já publicados. Mais estudos relacionando a dermatite atópica à vitamina D são desejáveis, tendo em vista a sua possível aplicabilidade como terapêutica auxiliar.

ABSTRACT Introduction: Atopic dermatitis (AD) is a chronic inflammatory multifactorial disease. Severe cases affect the quality of life. The pathogenesis is complex, marked by defective cellular immune system, exacerbation of Th2-type immune response and impaired skin barrier function. Studies suggest that vitamin D acts in the regulation of the innate and adaptive immune response, reduces the inflammatory process and improves the skin barrier. Our objective was to correlate vitamin D serum levels with the severity of AD. Material and Methods: Between 2015 and 2018 the authors evaluated 30 patients aged between 18 and 90 years old, under treatment at a Dermatology ambulatory clinic. Patients were divided into 2 groups: mild disease (only topical therapy) and moderate to severe disease (under immunosuppressive treatment). Results: Ten cases with mild AD and 20 with moderate to severe AD were selected. Among patients with mild disease, 20% had vitamin D deficiency. As for patients with moderate to severe disease, 40% had a deficient serum level of 25-hydroxyvitamin D. Comparing the average vitamin D in the two groups, there was no statistical difference between them. Conclusion: In the literature, the association between vitamin D levels and the severity of AD remain controversial, although some studies support this association. In this study, despite the proportion of patients with 25-hydroxyvitamin D deficiency being twice as high among patients with moderate to severe AD, there was no statistical significance between the groups, corroborating previous manuscripts. Clinical studies relating atopic dermatitis to vitamin D are warranted, considering its possible use as a concomitant therapy.

Abstract: Psoriasis is a chronic inflammatory disease that affects 1.3% of the Brazilian population. The most common clinical manifestations are erythematous, scaling lesions that affect both genders and can occur on any anatomical site, preferentially involving the knees, elbows, scalp and genitals. Besides the impact on the quality of life, the systemic nature of the disease makes psoriasis an independent risk factor for cardiovascular disease, especially in young patients with severe disease. By an initiative of the Brazilian Society of Dermatology, dermatologists with renowned clinical experience in the management of psoriasis were invited to form a work group that, in a partnership with the Brazilian Medical Association, dedicated themselves to create the Plaque Psoriasis Diagnostic and Treatment Guidelines. The relevant issues for the diagnosis (evaluation of severity and comorbidities) and treatment of plaque psoriasis were defined. The issues generated a search strategy in the Medline-PubMed database up to July 2018. Subsequently, the answers to the questions of the recommendations were devised, and each reference selected presented the respective level of recommendation and strength of scientific evidence. The final recommendations for making up the final text were worded by the coordinators.

Abstract: Psoriasis is a chronic disease, characterized by erythematous scaly lesions, presented in eight different forms: plaques, guttate, pustular, erythrodermic, inverse, nail and scalp psoriasis, and psoriatic arthritis. Its development depends on genetic factors, external stimulus and immune response alteration.1 Proinflammatory cytokines such as TNF-alpha, IL-12 and 23 may also be involved. In the worst cases, systemic complications linked to endothelial alterations may occur. A literature review was conducted for a better understanding of what roles VEGF (vascular endothelial growth factor) and ICAM-1 (intercellular adhesion molecule) have, among other cytokines, in systemic capillary leak syndrome, involved in erythrodermic and pustular psoriasis, the most unstable forms of the disease.

Abstract: Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis.

Bullous pemphigoid (BP) is an autoimmune, acquired, cutaneous disease caused by the production of autoantibodies against hemidesmosomes' components in the basement membrane. The estimated incidence in Europe ranges from 7 to 43 cases per million inhabitants per year. Several studies have reported an association between BP and neurological disorders (ND). Our cohort of Bullous pemphigoid and ND is the first in Brazil and showed a significantly high prevalence of neurological and/or psychiatric diseases, especially cerebrovascular accident (CVA) and dementia, in agreement with the prevalence reported in several studies published in the medical literature in recent years.

Os medicamentos antagonistas do fator de necrose tumoral alfa (TNF-α) estão sendo cada vez mais utilizados no tratamento de doenças inflamatórias e autoimunes. Efeitos adversos desses medicamentos vem sendo relatados, incluindo o desenvolvimento paradoxal de sarcoidose, principalmente com o uso do etanercepte. Apresentamos o primeiro relato de caso brasileiro de sarcoidose sistêmica induzida por etanercepte e uma revisão da literatura.

The antagonists of tumor necrosis factor alpha (TNF-α) are increasingly being used in the treatment of inflammatory and autoimmune diseases. Several adverse effects of these drugs have been reported, including the paradoxical development of sarcoidosis, especially with the use of etanercept. We present the first Brazilian case report of systemic sarcoidosis induced by etanercept and a literature review.

A psoríase é uma doença inflamatória crônica que pode afetar a pele e as articulações. Seu tratamento varia conforme a gravidade e inclui medicamentos tópicos e sistêmicos. Dentre os últimos estão os imunobiológicos, que têm como alvo a célula T. Relatamos um caso que demonstra a estreita relação entre a psoríase, o linfoma e os imunobiológicos.

Psoriasis is a chronic inflammatory disease that can affect skin and joints. Their treatment varies depending on the severity and includes topical and systemic. Among the latter are the immunobiological that target the T cell We report a case that demonstrates the close relationship between psoriasis, lymphoma and biologic therapies.

Relata-se o caso de um homem de 45 anos com dermatofitose superficial de longa data, tratado, inadvertidamente, com corticoide e antibiótico, com progressão subsequente para a forma profunda, conhecida como granuloma de Majocchi. O tratamento com terbinafina VO foi curativo

We report the case of a man of 45 with superficial dermatophytosis longtime inadvertently treated with antibiotics and corticosteroids with subsequent progression to the deep form, known as granuloma Majocchi. Treatment with orally terbinafine was successful

A síndrome de extravasamento capilar sistêmico é rara e causada por aumento da permeabilidade capilar. Várias etiologias estão envolvidas. No nosso serviço de Dermatologia, a principal é a psoríase instável. Diversos tratamentos são usados e muitos ainda estão em estudo. Nosso objetivo foi apresentar esta situação clínica potencialmente fatal que ocorre em nossa especialidade

The systemic capillary leak syndrome is rare and caused by increased capillary permeability. Several etiologies are involved. In our Department of Dermatology the main one is unstable psoriasis. Several treatments are used and many are still under study. Our objective was to present this potentially fatal medical condition that occurs in our specialty

Relatamos um caso de higroma cístico na face de uma criança de quatro meses de idade. Havia história de aumento de volume congênito na hemiface direita que involuiu consideravelmente após sinais de infecção. O higroma cístico ou linfangioma é uma malformação congênita rara do sistema linfático, presente ao nascimento em 50% dos casos. Localiza-se preferencialmente na região cervical e na face. Geralmente evolui com crescimento lento e progressivo, podendo comprimir e infiltrar estruturas adjacentes. Sua regressão espontânea ocorre em apenas 6% dos casos.

This report describes the case of a cystic hygroma on the face of a four-month old child. There was a history of congenital swelling of the right hemiface that decreased considerably following signs of infection. The cystic hygroma or lymphangioma is a rare congenital malformation of the lymphatic system that is present at birth in 50% of cases. It is usually located on the neck or face. It generally grows slowly and progressively and may compress and infiltrate adjacent structures. Its spontaneous regression occurs in only 6% of cases.

O Angioma em tufo é considerado um raro tumor vascular cutâneo adquirido. Caracteriza-se por máculas purpúrico-violáceas, dolorosas que podem evoluir para placas com pápulas angiomatosas. Tem natureza benigna, mas o envolvimento extenso é comum, gerando alteração funcional do membro afetado em caso de dor. Os autores relatam um caso de angioma em tufo associado à síndrome dolorosa miofascial, em que o elemento predisponente foi a presença deste tumor desde a infância. A dor local prejudicou a utilização da musculatura e possibilitou o surgimento da síndrome relatada. A realização de bloqueio anestésico de pontos-gatilhos no membro afetado, levou à melhora total dos sintomas.

Tufted angioma is a rare acquired vascular tumor. It is characterized by painful purplish macules that may progress to plaques containing angiomatous papules. The condition is benign; however, it often affects extensive areas of the skin, leading to functional disability of the affected limb if painful. The present report describes a case of a tufted angioma associated with myofascial pain syndrome in which the predisposing element was the presence of this tumor since childhood. Pain at the site of the lesion affected muscle use and led to the onset of the associated syndrome. Complete relief from symptoms was achieved by blocking the trigger points of the affected limb with anesthesia.

O pseudoxantoma elástico perfurante periumbilical (PEPP), ou elastose cálcica perfurante, é distúrbio raro e sua patogênese está associada a alteração da fibra elástica, tendo o trauma provável participação. Apresenta-se caso de paciente de 70 anos com quadro de máculas enegrecidas e pápulas encimadas por crostas na região periareolar e periumbilical há mais de dez anos. O exame histopatológico revelou fibras elásticas alteradas, repletas de cálcio, e formação de pertuito na derme com fibras elásticas degradadas no seu interior. O caso descrito corresponde ao PEPP, que nesta paciente também acomete área periareolar bilateralmente.

The periumbilical perforating pseudoxanthoma elasticum or perforating calcific elastosis is a rare disorder and its pathogenesis is associated with the alteration of elastic fibers, which may probably result from trauma. We present the case of a 70-year-old woman with long-time blackish maculas and keratotic surface papules on the periareolar and periumbilical regions. The histopathological examination revealed altered elastic fibers, replete with calcium and formation of a path in the dermis with elastic fibers degraded in their interior, confirming the clinical diagnosis.

A calcinose idiopática é uma doença rara, caracterizada pelo depósito de sais de cálcio na derme. Possui diversas apresentações clínicas. Em pacientes com Síndrome de Down, ocorre uma rara associação com lesões de calcinose diopática semelhantes a milium.

Idiopathic calcinosis is a rare disease characterized by abnormal deposit of calcium salts on the dermis. It has several clinical presentations. In patients suffering from Down Syndrome, there is a rare association with idiopathic calcinosis lesions similar to milium.