Skin manifestations associated with systemic diseases - Part I

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.

Case for diagnosis. Ulcerated lesions: a diagnostic challenge in Behçet's syndrome

Abstract This study reports the clinical case of a 42-year-old patient with ulcerated lesions who was followed up by general practitioners with the diagnosis of recurrent cellulitis. However, when referred to the Dermatology division a diagnosis of Behçet's syndrome was established based on clinical criteria. Although there are defined clinical criteria for this syndrome, sometimes its diagnosis can be challenging, due to lack of knowledge of the disease and extremely heterogeneous clinical phenotype. The authors highlight the potential difficulties in establishing the diagnosis considering the multiple clinical findings during the investigation process, contributing to the risk of increased morbidity and mortality.

Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology

Abstract: Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. For each of these dermatoses, a literature review covering all therapeutic options was performed. A flowchart, based on the experience and joint discussion among the authors of this consensus, was constructed to provide treatment orientation for these diseases in Brazil. In summary, in the localized, low-risk or non-severe forms, drugs that have immunomodulatory action such as dapsone, doxycycline among others may be a therapeutic option. Topical treatment with corticosteroids or immunomodulators may also be used. Systemic corticosteroid therapy continues to be the treatment of choice for severe forms, especially those involving ocular, laryngeal-pharyngeal and/or esophageal mucosal involvement, as may occur in mucous membrane pemphigoid and epidermolysis bullosa acquisita. Several immunosuppressants are used as adjuvant alternatives. In severe and recalcitrant cases, intravenous immunoglobulin is an alternative that, while expensive, may be used. Immunobiological drugs such as rituximab are promising drugs in this area. Omalizumab has been used in bullous pemphigoid.

Granuloma annulare on the palms in an elderly patient

Abstract: Granuloma annulare is a relatively common, idiopathic, benign inflammatory dermatosis, with a varied clinical presentation that often makes diagnosis difficult. It mainly affects the extremities, such as the dorsa of the hands and feet, forearms and legs. Palmar and plantar regions are generally spared. It occurs mainly in young female patients. The presentation of the palmar variant in an elderly patient is a rarity.

Morbihan disease: a therapeutic challenge

Abstract: Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Pemphigus foliaceus with pustular presentation in a patient with psoriasis

Abstract Pemphigus foliaceus is a chronic autoimmune disease of the skin, clinically characterized by scaly and crusty cutaneous erosions involving the seborrheic areas. The patient can eventually become erythrodermic. There are reports of atypical cases of pemphigus foliaceus with pustules and neutrophils, and clinical differentiation from generalized pustular psoriasis of von Zumbusch is difficult. We report the case of a 55-year-old man with a history of psoriasis vulgaris that has developed pemphigus foliaceus with pustules, triggered by withdrawal of systemic corticosteroids. This is the first report associating this atypical form of pemphigus with psoriasis, suggesting that an overlap with generalized pustular psoriasis can occur.

Histiocytoid Sweet's syndrome presenting with annular erythematous plaques

Abstract Histiocytoid Sweet's Syndrome is a rare inflammatory disease described in 2005 as a variant of the classical Sweet's Syndrome (SS). Histopathologically, the dermal inflammatory infiltrate is composed mainly of mononuclear cells that have a histiocytic appearance and represent immature myeloid cells. We describe a case of Histiocytoid Sweet's Syndrome in an 18-year-old man. Although this patient had clinical manifestations compatible with SS, the cutaneous lesions consisted of erythematous annular plaques, which are not typical for this entity and have not been described in histiocytic form so far. The histiocytic subtype was confirmed by histopathological analysis that showed positivity for myeloperoxidase in multiple cells with histiocytic appearance.

Gianotti-Crosti syndrome: a case report of a teenager

Abstract Gianotti-Crosti syndrome is a rare disease characterized by acral papular eruption with symmetrical distribution. It is a benign and self-limited disease; the symptoms disappear after two to eight weeks, without recurrences or scars. Skin lesions are usually asymptomatic. Prodrome might occur, suggesting upper respiratory infection, or constitutional symptoms. Diagnosis is eminently clinical, and this disease is associated with viral infections. Due to its rarity and low occurrence in adolescents and adults, we report a case of Gianotti-Crosti syndrome of a teenager.

Lichen sclerosus associated with localized scleroderma: dermoscopy contribution

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.

Geographic tongue and psoriasis: clinical, histopathological, immunohistochemical and genetic correlation - a literature review

Abstract: Geographic tongue is a chronic, inflammatory, and immune-mediated oral lesion of unknown etiology. It is characterized by serpiginous white areas around the atrophic mucosa, which alternation between activity, remission and reactivation at various locations gave the names benign migratory glossitis and wandering rash of the tongue. Psoriasis is a chronic inflammatory disease with frequent cutaneous involvement and an immunogenetic basis of great importance in clinical practice. The association between geographic tongue and psoriasis has been demonstrated in various studies, based on observation of its fundamental lesions, microscopic similarity between the two conditions and the presence of a common genetic marker, human leukocyte antigen (HLA) HLA-C*06. The difficulty however in accepting the diagnosis of geographic tongue as oral psoriasis is the fact that not all patients with geographic tongue present psoriasis. Some authors believe that the prevalence of geographic tongue would be much greater if psoriatic patients underwent thorough oral examination. This study aimed to develop a literature review performed between 1980 and 2014, in which consultation of theses, dissertations and selected scientific articles were conducted through search in Scielo and Bireme databases, from Medline and Lilacs sources, relating the common characteristics between geographic tongue and psoriasis. We observed that the frequency of oral lesions is relatively common, but to establish a correct diagnosis of oral psoriasis, immunohistochemical and genetic histopathological analyzes are necessary, thus highlighting the importance of oral examination in psoriatic patients and cutaneous examination in patients with geographic tongue.

Síndrome de Poland: dois relatos de caso com suas respectivas condutas cirúrgicas / Poland's syndrome: two case reports with their respective surgical procedures

A Síndrome de Poland é uma anomalia congênita de baixa incidência, que afeta em média 1:32000 nascidos vivos e tem causa idiopática. Essa condição é caracterizada pela atrofia ou ausência do músculo peitoral maior, mas pode acometer também outros músculos, como o peitoral menor e serrátil. Nessa síndrome pode ocorrer associação de outros fenômenos, como a sindactilia ipsilateral, hipoplasia da mão, alterações ósseas e deformidade de Sprengel, na qual a escápula passa a ser menor e alada. No presente estudo, relatamos dois casos de Síndrome de Poland com base na revisão de prontuários. No primeiro caso, percebemos uma atrofia muscular na mama direita em que foi necessária a utilização de dois expansores para fazer a reconstrução, um na mama direita e outro na região toracoabdominal, em virtude de atrofia tecidual entre a pele e o osso. Em sequência, foi colocada a prótese mamária definitiva de silicone e foi feita a correção da atrofia tecidual. Já no segundo caso, não houve a necessidade de colocação de expansores. Em cada caso foi usada uma maneira de correção, uma vez que, nesta síndrome, não há um método único de tratar o paciente, devendo cada caso ser estudado individualmente. Com o estudo, é possível notar que a Síndrome de Poland não causa uma patologia de fato, já que na ausência do músculo peitoral maior não há comprometimentos motores, o que ocorre é um comprometimento estético(AU)

Poland 's syndrome is a congenital abnormality of low incidence, which affects roughly 1: 32,000 live births and is idiopathic. This condition is characterized by atrophy or absence of the pectoralis major muscle, but can also affect other muscles such as the pectoralis minor and serratus. This syndrome can occur in association with such other phenomena as ipsilateral syndactyly, hand hypoplasia, bone changes, and Sprengel's deformity, in which the scapula becomes smaller and winged. In the present study we report two cases of Poland's syndrome based on chart review. In the first case there was muscular atrophy in the right breast, where the use of two expanders was required for the reconstruction, one on the right breast and the other in the thoracoabdominal region, due to tissue atrophy between the skin and the bone. Subsequently, the final silicone breast implant was placed and correction of tissue atrophy was performed. In the second case there was no need for using expanders. In each case a different way of correction was used, since in this syndrome there is no single method to treat the patient, and each case should be studied individually. From this study one can see that Poland's Syndrome does not cause an actual pathology, as the absence of the pectoralis major does not bring any motor compromising; what happens is aesthetic compromising(AU)

Percepção dos cuidadores sobre as alterações de deglutição causadas pela demência / Perception of caregivers about swallowing disorders caused by dementia

Objetivo: Identificar o grau de conhecimento dos cuidadores e/ou familiares de pacientes com demência sobre a doença e as alterações de deglutição envolvidas por meio de questionário específico. Material e Métodos: Foram entrevistados 150 acompanhantes de pacientes com demência atendidos em um hospital público terciário. Foram realizadas questões acerca do conhecimento do cuidador sobre o diagnóstico de demência dos pacientes e da atuação fonoaudiológica nestes casos. Coletadas as informações,realizou-se a análise descritiva dos dados. Resultados: A demência de Alzheimer foi diagnosticada em 58,7% dos pacientes envolvidos na pesquisa. Os cuidadores eram, em98% dos casos, familiares dos pacientes, 82,7% do gênero feminino, e 48,7% filhos; 52,7% dos cuidadores descreveram perda de memória como manifestação do quadro clínico do paciente que cuidavam; 56% dos entrevistados relataram conhecer a existência do profissional fonoaudiólogo, e 79,3%não sabiam em que o fonoaudiólogo poderia auxiliar na assistência do paciente com demência. Na percepção das alterações fonoaudiológicas 36,6% observavam comprometimento da deglutição. Conclusão: Os conhecimentos dos cuidadores, acerca da demência e das alterações de deglutição, foram precários. É essencial que os cuidadores recebam orientações específicas para identificar os sinais de alterações de deglutição e favorecer uma alimentação segura para o paciente e evitar complicações futuras como pneumonias aspirativas.

Objective: To identify the degree of knowledge of caregivers and/or family members of patients with dementia about the disease and swallowing alterations. Materials and Methods:We interviewed 150 caregivers of patients with dementia treated in a tertiary health care public hospital. A specific questionnaire was used to collect information about the caregiver’s knowledge on diagnosis of dementia and on the role of speech language therapy in these cases. The data were analyzed descriptively. Results: Alzheimer’s dementiawas diagnosed in 58.7% of patients included in the research. Caregivers were family members in 98% of the cases; 82.7% were female; and 48.7% sons/daughters. A total of 52.7% of caregivers reported that memory loss was the clinical manifestation of the patient who they took care for; 56% reported knowing the existence of a professional speech language therapist, and 79.3% did not know how the speech language therapist could help in the care of the patient with dementia. With regard to the perception of speech language, 36.6% observed impaired swallowing. Conclusion: The knowledge of caregivers about dementia and swallowing disorders was found to be poor. It is essential that caregivers receive specific guidelines to identify signs of swallowing disorders and assist in the patient’s safe eating in order to prevent future complications such as aspiration pneumonia.

Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy

Abstract: Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

Identificação da microbiota da cavidade oral de equinos / Oral cavity microbiota identification of horses / Identificación de la microbiota de la cavidad oral de equinos

Este trabalho descreve a recuperação e a identificação de bactérias da microbiota oral de equinos sadios provenientes da Sociedade Rural de Umuarama-PR e de centros de treinamento de Quarto de Milha da região. Foram coletados espécimes orais de 48 animais adultos de ambos os sexos, utilizando suabe estéril que foram semeados em ágar base acrescido de 5-8% de sangue ovino desfibrinado. As cepas isoladas foram identificadas segundo as suas características morfocoloniais, morfotinturiais e testes bioquímicos. Foram isolados a partir desses animais cocos gram-positivos (Staphylococcus spp., Streptococcus spp. E Nocardia spp.) e gram-negativos (Moraxella spp.) além de bastonetes gram-negativos, residentes das regiões periodontal e terço médio da lingua. Os principais isolados bacterianos das amostras periodontais foram Staphylococcus spp. Em 81,25% (39/48) das amostras, seguido por Streptococcus spp. Em 41,67% (20/48) das amostras. Os achados derivados das amostras da lingua mostraram maior colonização de Streptococcus spp. Comparada aos Staphylococcus spp. Os resultados obtidos representaram contribuição original para o conhecimento da microbiota oral de equinos, tendo significado para a microbiologia comparada.

This paper describes the recovery and identification of bacteria from the oral microbiota of healthy horses from the Rural Society (Sociedade Rural) in Umuarama-PR and Quarter Horse training centers in the region. Oral specimens were collected from 48 adult animals of both sexes, using sterile swabs plated on blood agar. Isolates were identified according to their morpho-colonial, staining and biochemical test characteristics. Gram-positive (Staphylococcus spp. and Streptococcus spp., Nocardia spp.) and gram-negative (Moraxella spp.) cocci, as well as periodontal rod cells were isolated from the periodontal and middle third portion of the tongue. The main bacterial isolates from periodontal samples were Staphylococcus spp., found in 81.25% (39/48) samples, followed by Streptococcus spp. in 41.67% (20/48) samples. The findings derived from tongue samples presented higher Streptococcus spp colonization. Compared to Staphylococcus spp., the results represent an original contribution to the knowledge of horse oral microbiota, with significance to compared microbiology.

Esta investigación describe la recuperación y la identificación de bacterias de la microbiota oral en equinos sanos provenientes de la Sociedad Rural de Umuarama-PR y de centros de entrenamiento de Cuarto de Milla de la región. Se ha recolectado muestras orales de 48 animales adultos de ambos sexos, utilizando hisopos estériles que fueron sembrados en ágar base añadido de 5-8% de sangre ovino desfibrinado. Las cepas aisladas fueron identificadas segundo sus características morfo coloniales, morfo tintúrales y pruebas bioquímicas. Se aislaron a partir de esos animales cocos gran positivos (Staphylococcus spp., Streptococcus spp. Y Nocardia spp.) y gran negativos (Moraxella spp.), además de bastones gran negativos, residentes de las regiones periodontal y medio de la lengua. Los principales aislados bacterianos de las muestras periodontales fueron Staphylococcus spp. En 81,25% (39/48) de las muestras, seguido por Streptococcus spp. En 41,67% (20/48) de las muestras. Los hallazgos derivados de las muestras de la lengua presentaron mayor colonización de Streptococcus spp. Comparada a los Streptococcus spp. Los resultados obtenidos representan una contribución original al conocimiento de la microbiota oral de equinos, que tienen significado para la microbiología comparada.

Plant regulators and invertase activity in sugarcane at the beginning of the harvest season / Reguladores vegetais e atividade de invertases em cana de açúcar no início da safra

Invertases play an essential role in partitioning photosynthates between storage and growth. The objective of this study was to evaluate the activity of acid and neutral invertases and the role they play in controlling the accumulation of sucrose in sugarcane as a result of the application of plant regulators in the beginning of the cropping season.A randomized block experimental design was adopted, with five replicates.The treatments consisted in the application of three plant regulators of the class of growth inhibitors (Sulfomethuron-methyl - 20g ha-1, Glyphosate - 0.4L ha-1, and Compounds from organic carboxylic radicals + Glyphosate - 1L ha-1 + 0.15L ha-1), in addition to a control (natural ripening).The acid and neutral invertase levels are affected in different ways and intensities, due to the active principle used as ripening agent and to the weather conditions.In sugarcane variety RB85-5453, with the conditions described in this experiment, it is suggested high levels of soluble acid invertase in relation to levels of neutral invertase; however, the first was characterized by high sucrose content in the stalks.Inverse correlation could be established for sugarcane variety RB85-5453 between soluble acid invertase levels and effective sucrose accumulation in the stalks.

Invertases desempenham um papel essencial no particionamento de fotossintatos entre armazenamento e crescimento. O objetivo deste estudo foi avaliar a atividade das enzimas invertases ácida e alcalina (neutra) e o papel que desempenham no controle do acúmulo de sacarose em cana-de-açúcar, como resultado da aplicação de reguladores vegetais no início da época de colheita. O delineamento experimental utilizado foi em blocos ao acaso, com cinco repetições. Os tratamentos consistiram na aplicação de três reguladores vegetais da classe dos inibidores de crescimento (Sulfometuron-metil - 20g ha-1, Glifosato - 0,4L ha-1, e Compostos de radicais carboxílicos orgânicos + Glifosato - 1L ha-1 + 0,15L ha-1), além do controle (maturação natural). Os níveis das invertases ácida e neutra são afetados de maneiras e intensidades distintas, devido ao princípio ativo utilizado como agente de maturação e das condições meteorológicas. Para a variedade RB85-5453, nas condições descritas neste experimento, sugerem-se níveis elevados de SAI em relação à NI;no entanto, a primeira foi caracterizada por um elevado teor de sacarose nos colmos. Correlação inversa pode ser estabelecida entre a atividade da invertase ácida e o acúmulo de sacarose.

Identificação da microbiota da cavidade oral de equinos / Oral cavity microbiota identification of horses / Identificación de la microbiota de la cavidad oral de equinos

Este trabalho descreve a recuperação e a identificação de bactérias da microbiota oral de equinos sadios provenientes da Sociedade Rural de Umuarama-PR e de centros de treinamento de Quarto de Milha da região. Foram coletados espécimes orais de 48 animais adultos de ambos os sexos, utilizando suabe estéril que foram semeados em ágar base acrescido de 5-8% de sangue ovino desfibrinado. As cepas isoladas foram identificadas segundo as suas características morfocoloniais, morfotinturiais e testes bioquímicos. Foram isolados a partir desses animais cocos gram-positivos (Staphylococcus spp., Streptococcus spp. E Nocardia spp.) e gram-negativos (Moraxella spp.) além de bastonetes gram-negativos, residentes das regiões periodontal e terço médio da lingua. Os principais isolados bacterianos das amostras periodontais foram Staphylococcus spp. Em 81,25% (39/48) das amostras, seguido por Streptococcus spp. Em 41,67% (20/48) das amostras. Os achados derivados das amostras da lingua mostraram maior colonização de Streptococcus spp. Comparada aos Staphylococcus spp. Os resultados obtidos representaram contribuição original para o conhecimento da microbiota oral de equinos, tendo significado para a microbiologia comparada...

This paper describes the recovery and identification of bacteria from the oral microbiota of healthy horses from the Rural Society (Sociedade Rural) in Umuarama-PR and Quarter Horse training centers in the region. Oral specimens were collected from 48 adult animals of both sexes, using sterile swabs plated on blood agar. Isolates were identified according to their morpho-colonial, staining and biochemical test characteristics. Gram-positive (Staphylococcus spp. and Streptococcus spp., Nocardia spp.) and gram-negative (Moraxella spp.) cocci, as well as periodontal rod cells were isolated from the periodontal and middle third portion of the tongue. The main bacterial isolates from periodontal samples were Staphylococcus spp., found in 81.25% (39/48) samples, followed by Streptococcus spp. in 41.67% (20/48) samples. The findings derived from tongue samples presented higher Streptococcus spp colonization. Compared to Staphylococcus spp., the results represent an original contribution to the knowledge of horse oral microbiota, with significance to compared microbiology. KEYWORDS: Horses. Microbiota. Oral. Staphylococcus spp. Streptococcus spp...

Esta investigación describe la recuperación y la identificación de bacterias de la microbiota oral en equinos sanos provenientes de la Sociedad Rural de Umuarama-PR y de centros de entrenamiento de Cuarto de Milla de la región. Se ha recolectado muestras orales de 48 animales adultos de ambos sexos, utilizando hisopos estériles que fueron sembrados en ágar base añadido de 5-8% de sangre ovino desfibrinado. Las cepas aisladas fueron identificadas segundo sus características morfo coloniales, morfo tintúrales y pruebas bioquímicas. Se aislaron a partir de esos animales cocos gran positivos (Staphylococcus spp., Streptococcus spp. Y Nocardia spp.) y gran negativos (Moraxella spp.), además de bastones gran negativos, residentes de las regiones periodontal y medio de la lengua. Los principales aislados bacterianos de las muestras periodontales fueron Staphylococcus spp. En 81,25% (39/48) de las muestras, seguido por Streptococcus spp. En 41,67% (20/48) de las muestras. Los hallazgos derivados de las muestras de la lengua presentaron mayor colonización de Streptococcus spp. Comparada a los Streptococcus spp. Los resultados obtenidos representan una contribución original al conocimiento de la microbiota oral de equinos, que tienen significado para la microbiología comparada...

Systemic sarcoidosis induced by etanercept: first Brazilian case report / Sarcoidose sistemica induzida por etanercepte: primeiro relato de caso brasileiro

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.

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.

Diffuse systemic sclerosis with bullous lesions without systemic manifestations / Esclerose sistemica difusa bolhosa sem acometimentos sistemicos

Here, we describe an atypical case of systemic sclerosis in its diffuse cutaneous form with acute and rapid progression of the cutaneous condition, without any systemic manifestations and the infrequent formation of bullae, showing the importance of diagnosis and early treatment in such cases. This case also shows that special measures should be taken for bullous cutaneous lesions and ulcerations resulting from serious sclerosis, which are entry points and increase morbidity and risk of death. Other prognostic factors include age, ESR and renal and pulmonary involvement. Capillaroscopies can be useful predictors of greater severity of systemic scleroderma, revealing a greater link with systemic, rather than cutaneous, involvement.

Descrevemos um caso atípico de esclerose sistêmica em sua forma cutânea difusa com instalação aguda e rápida progressão do quadro cutâneo sem qualquer acometimento sistêmico e a infrequente formação de bolhas, demostrando a importância do diagnóstico e tratamento precoce frente a casos semelhantes e mostrando, com a experiência deste caso, que cuidados especiais devem ser tomados com as lesões cutâneas bolhosas e as ulcerações decorrentes da grave esclerose que são portas de entrada e aumentam a morbidade e risco de morte. Outros fatores prognósticos descritos são idade, VHS e envolvimento pulmonar e renal. A capilaroscopia pode ser preditor de maior gravidade da esclerodermia sistêmica, guardando maior relação com o envolvimento sistêmico do que cutâneo.

Dermatoscopic findings of urticaria pigmentosa / Achados dermatoscopicos na urticaria pigmentosa

Mastocytosis is a rare disease characterized by proliferation and accumulation of mast cells in various organs. The maculopapular cutaneus mastocytosis is divided into three subtypes: papular/plaque variant, urticaria pigmentosa and eruptive macular telangiectasia perstans. Dermoscopic may help to better characterize the different forms of cutaneus mastocytosis. We report a 55 year-old female with urticaria pigmentosa and its dermoscopy.

Mastocitose é uma doença rara caracterizada pela proliferação e acúmulo de mastócitos em vários órgãos. A mastocitose cutanea maculopapular divide-se em três subtipos: variante máculo-papular, urticária pigmentosa e telangiectasia macular eruptiva perstans. A dermatoscopia pode ajudar a caracterizar melhor as diferentes formas de mastocitose cutânea. Relatamos um caso de paciente feminina de 55 anos com urticária pigmentosa e sua dermatoscopia.