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BACKGROUND: Brief erythematous-papular skin rashes suggest the diagnosis of urticaria; However, it may be another type of dermatitis, and complementary examinations must be carried out to establish its diagnosis. CASE REPORT: 53-year-old female patient, diagnosed in 2016 with diffuse large B cell lymphoma, in complete remission. Since 2010, he has had episodes of erythematous-papular lesions lasting 24-36 hours. He received antihistamines, corticosteroids and omalizumab without clinical improvement. The ANA determination was positive (1/320), nuclear mitotic pattern. The skin biopsy was compatible with dermatitis herpetiformis. The study of celiac and locus antibodies showed positivity for HLA-DQ2 and DQ2.5 in heterozygosity. The diagnosis of dermatitis herpetiformis was established. Treatment consisted of a gluten-free diet and prescription of dapsone, with satisfactory results. CONCLUSION: It is important to establish the differential diagnosis of patients with chronic urticaria who do not respond to the reference treatment, in addition to carrying out a thorough clinical examination and physical examination before starting treatment and relying on a multidisciplinary team to establish an accurate diagnosis and treatment. appropriate. Due to the side effects of dapsone, subsequent follow-up of patients is essential.

ANTECEDENTES: Los exantemas cutáneos eritemato-papulares de breve duración sugieren el diagnóstico clínico de urticaria; no obstante, puede tratarse de otro tipo de dermatitis, y para establecer el diagnóstico deben llevarse a cabo exploraciones complementarias. REPORTE DE CASO: Paciente femenina de 53 años, diagnosticada en 2016 con linfoma difuso de células B grandes, en remisión completa. Desde el 2010 manifestó episodios de lesiones eritemato-papulosas, de 24-36 horas de duración. Recibió antihistamínicos, corticoides y omalizumab sin mejoría clínica. La determinación de ANA resultó positiva (1/320), con patrón mitótico nuclear. La biopsia cutánea fue compatible con dermatitis herpetiforme. El estudio de anticuerpos de celiaquía y locus mostró positividad para HLA-DQ2 y DQ2.5 con heterocigosis. Se estableció el diagnosticó de dermatitis herpetiforme. El tratamiento consistió en dieta exenta de gluten y prescripción de dapsona, con resultados satisfactorios. CONCLUSIÓN: Es importante establecer el diagnóstico diferencial de pacientes con urticaria crónica que no responden al tratamiento de referencia, además de efectuar el examen clínico y la exploración física exhaustivos antes de iniciar el protocolo, y apoyarse de un equipo multidisciplinario para establecer el diagnóstico certero y tratamiento adecuado. Debido a los efectos secundarios de la dapsona, es imprescindible el seguimiento posterior de los pacientes.

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La dermatitis herpetiforme es una enfermedad autoinmune que se caracteriza por la presencia de lesiones vesiculares y prurito en la superficie extensora de las extremidades, nalgas y parte baja de la espalda. Se presenta el caso clínico de una mujer de 21 años, natural y procedente de Iquitos, que presentó múltiples vesículas dolorosas, pruriginosas, de base eritematosa y purulentas en ambos codos durante un mes. Posteriormente, estas lesiones se diseminaron a ambas rodillas y se agregó prurito intenso. La paciente experimentó períodos alternantes de remisión y exacerbación. La correlación del cuadro clínico, resultados serológicos y de biopsia, junto con respuesta terapéutica a la dapsona confirmaron el diagnóstico de dermatitis herpetiforme, con una evolución favorable y remisión de la enfermedad.

Dermatitis herpetiforme is an autoimmune disease characterized by the presence of vesicular lesions and itching on the extensor surface of the limbs, buttocks, and lower back. The clinical case of a 21-year-old woman, a native of and from Iquitos, is presented. She presented multiple painful, itchy vesicles with an erythematous and purulent base on both elbows for a month. Subsequently, these lesions spread to both knees, and intense itching was added. The patient experienced alternating periods of remission and exacerbation. The correlation of the clinical picture, serological and biopsy results, along with the therapeutic response to dapsona, confirmed the diagnosis of dermatitis herpetiforme, with a favorable evolution and remission of the disease.

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Resumen: Introducción: la dermatitis herpetiforme (DH) es una enfermedad cutánea inflamatoria que cursa en empujes y remisiones. Es una manifestación extraintestinal de la enfermedad celíaca (EC), de la que puede ser la primera manifestación clínica(1). La enfermedad celíaca es una enfermedad sistémica autoinmune, asociada con enteropatía, desencadenada por la ingesta de gluten en individuos genéticamente predispuestos(2-4). Se ha demostrado una asociación con el HLA DQ2 y HLA DQ8 en ambas enfermedades(5). Objetivo: mostrar las características de los pacientes con DH y EC que se asisten en las policlínicas de dermatología y gastroenterología del Hospital Universitario Dr. Manuel Quintela. Metodología: se realizó un estudio descriptivo, observacional, retrospectivo y longitudinal de una serie de casos. Criterios de inclusión: pacientes asistidos en servicios de dermatología y gastroenterología del Hospital de Clínicas, con diagnóstico de DH confirmado con histopatología y/o inmunofluorescencia directa; desde julio de 2000 a junio de 2018 inclusive. Quedaron excluidos aquellos pacientes en los que no se pudo acceder al estudio histopatológico de piel. Resultados: incluimos un total de 15 pacientes, 9 de sexo masculino y 6 de sexo femenino. La edad media al diagnóstico de DH fue de 49 años. 4 pacientes recibieron tratamiento con dapsona, con rápida respuesta de la dermatosis, sin efectos adversos graves. En 13 pacientes la DH fue el síntoma guía para el diagnóstico de EC. 5 pacientes con EC asintomática, 6 con síntomas clásicos, 4 con síntomas no clásicos.

Summary: Introduction: dermatitis herpetiformis (DH) is an inflammatory cutaneous disease comprising relapses and remissions. It is an extraintestinal manifestation of coeliac disease, which can even be its first clinical manifestation. Coeliac disease (CD) is an autoimmune systemic disease, associated with enteropathy, that is induced by the intake of gluten in genetically predisposed individuals. In both diseases an association between HLA DQ2 and HLA DQ8 has been proved. Objective: to present the characteristics of patients with Dermatitis herpetiformis (DH) and Coeliac disease (CD) who are assisted in the dermatology and gastroenterology polyclinics at the "Dr. Manuel Quintela" University Hospital. Methodology: descriptive, observational, retrospective and longitudinal study of a series of cases. Inclusion criteria: patients assisted at the dermatology and gastroenterology services of the University Hospital, with a diagnosis of Dermatitis herpetiformis (DH) confirmed by histopathology and/or direct immunofluorescence (DIF), from July, 200 to June 2018 inclusive. Patients whose skin histopathological exam could not be accessed were excluded from the study. Results: 15 patients were included, 9 male and 6 female. Average age upon diagnosis of DH is 49 years old. 4 patients were treated with dapsona, evidencing a fast response to dermatosis, and no severe adverse effects. In 13 patients, DH was the guiding symptom to diagnose coeliac disease. However, 5 patients had asymptomatic CD, 6 presented classical symptoms and 4 non-classical symptoms.

Resumo: Introdução: a dermatite herpetiforme (DH) é uma doença inflamatória da pele que ocorre em impulsos e remissões. É uma manifestação extra-intestinal da doença celíaca (DC), da qual pode ser a primeira manifestação clínica. A doença celíaca é uma doença autoimune sistêmica associada à enteropatia, desencadeada pela ingestão de glúten em indivíduos geneticamente predispostos. Uma associação com HLA DQ2 e HLA DQ8 foi demonstrado em ambas as doenças. Objetivo: mostrar as características dos pacientes com HD e DC atendidos nas policlínicas dermatológicas e gastroenterológicas do "Dr. Manuel Quintela". Metodologia: foi realizado um estudo descritivo, observacional, retrospectivo e longitudinal de uma série de casos. Critérios de inclusão: pacientes atendidos nos serviços de dermatologia e gastroenterologia do Hospital de Clínicas, com diagnóstico de HD confirmado pela histopatologia e / ou imunofluorescência direta; de julho de 2000 a junho de 2018 inclusive. Foram excluídos os pacientes em que não foi possível acessar o estudo histopatológico da pele. Resultados: incluímos um N total de 15 pacientes, 9 homens e 6 mulheres. A idade média no diagnóstico de DH é de 49 anos. 4 pacientes receberam tratamento com dapsona, com resposta rápida da dermatose, sem efeitos adversos graves. Em 13 pacientes, a DH foi o sintoma norteador para o diagnóstico de DC. 5 pacientes com DC assintomática, 6 com sintomas clássicos, 4 com sintomas não clássicos.

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Resumen La enfermedad celíaca es un trastorno sistémico con base autoinmune, secundario a la exposición continua al gluten en personas con susceptibilidad genética, que lleva a desarrollo de anticuerpos específicos, afectando principalmente el intestino delgado con la subsecuente enteropatía. Las manifestaciones clínicas incluyen síntomas digestivos y manifestaciones extra intestinales. En este último grupo se destacan las manifestaciones dermatológicas, específicamente la dermatitis herpetiforme, la cual es considerada específica de la enfermedad celíaca. Clínicamente, se presenta como un exantema papulovesicular pruriginoso. El diagnóstico se basa en la biopsia de piel con inmunofluorescencia directa y la presencia de anticuerpos anti-transglutaminasa tisular 2 y solo en casos específicos se debe complementar con otros estudios. El tratamiento se basa en dieta libre de gluten, que mejora la sintomatología, controla y previene las complicaciones de la enfermedad celíaca, como las neoplasias digestivas y el desarrollo de otras enfermedades autoinmunes.

Abstract Celiac disease is a systemic disorder with an autoimmune basis, secondary to continuous exposure to gluten in people with genetic susceptibility, which leads to the development of specific antibodies mainly affecting the small intestine with subsequent enteropathy. The clinical manifestations include digestive symptoms and extra intestinal manifestations. In this last group, the dermatological manifestations stand out, specifically dermatitis herpetiformis, which is considered specific to Celiac disease. It presents clinically as a pruritic papulovesicular rash. The diagnosis is based on a skin biopsy with direct immunofluorescence and the presence of anti-tissue transglutaminase 2 antibodies, only in specific cases it should be complemented with other studies. Treatment is based on a gluten-free diet, which improves symptoms, controls and prevents complications of Celiac disease, such as digestive neoplasms and the development of other autoimmune diseases.

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ABSTRACT BACKGROUND: Dermatitis herpetiformis (DH) is considered a skin celiac disease (CD). The individuals can be seen by primary care professionals or by dermatologists that could refer the patient to a gastroenterologist. OBJECTIVE: The study aimed to investigate the clinical profile of patients diagnosed with DH and referred to a gastroenterologist and evaluate the treatment response. METHODS: We retrospectively studied patients with DH referred to the same gastroenterologist at a private office in Curitiba, Brazil, between January 2010 to December 2019. We included adult patients with a confirmed DH diagnosis. Symptoms, clinical signs, laboratory and histological data, as well as treatment response, were collected. RESULTS: Thirty-three patients were studied (60.6% women, mean age at diagnosis 40.8±12.61 years). The median delay for DH diagnosis was four years. Skin involvement was mild in 33.3%, moderate in 18.2%, and severe in 48.5%. The more frequent gastrointestinal complaints were abdominal distension (78.8%), flatulence (75.7%), and gastroesophageal reflux (51.5%). Depression and anxiety were observed in 81.8% and anemia in 51.1%. A higher prevalence of bone disorders was associated with higher age at DH diagnosis (P=0.035). Duodenal biopsy showed changes in all patients. Improvement after treatment only with a gluten-free diet (GFD) plus dapsone was verified in 81.2%. CONCLUSION: Patients with DH referred to a gastroenterologist showed a high frequency of gluten intolerance and systemic complaints. Duodenal histological alterations were found in all the cases. The treatment based on GFD plus dapsone was effective in most patients.

RESUMO CONTEXTO: A dermatite herpetiforme (DH) é considerada como a doença celíaca (DC) da pele. Os pacientes podem ser atendidos por profissionais do atendimento primário ou por dermatologistas que podem encaminhar o paciente a um gastroenterologista. OBJETIVO: Os objetivos do estudo foram investigar o perfil clínico dos pacientes com diagnóstico de DH encaminhados a um gastroenterologista e avaliar a resposta ao tratamento. MÉTODOS: Foram investigados retrospectivamente pacientes com DH encaminhados ao mesmo gastroenterologista em consultório particular em Curitiba, Brasil, entre janeiro de 2010 a dezembro de 2019. Foram incluídos pacientes adultos com diagnóstico confirmado de DH. Dados sobre sintomas e sinais clínicos, dados laboratoriais, histológicos e resposta ao tratamento foram coletados. RESULTADOS: Foram estudados 33 pacientes (60,6% mulheres, média de idade 40,8±12,61 anos). O atraso médio para o diagnóstico de DH foi de 4 anos. O envolvimento cutâneo foi considerado leve em 33,3%, moderado em 18,2% e grave em 48,5%. As queixas gastrointestinais mais frequentes foram distensão abdominal (78,8%), flatulência (75,7%) e refluxo gastroesofágico (51,5%). Depressão e ansiedade foram observadas em 81,8% e anemia em 51,1%. Maior prevalência de alterações ósseas foi associada à maior idade ao diagnóstico de DH (P=0,035). A biópsia duodenal mostrou alterações em todos os pacientes. A melhora após o tratamento apenas com dieta sem glúten e/ou dapsona foi verificada em 81,2%. CONCLUSÃO: Pacientes com DH encaminhados ao gastroenterologista apresentaram alta frequência de queixas gastrointestinais e sistêmicas. Alterações histológicas duodenais foram encontradas em todos os casos. O tratamento à base de dieta sem glúten e/ou dapsona foi eficaz na maioria dos pacientes.

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Dermatitis herpetiformis is an autoimmune chronic blistering disease, considered a skin manifestation of celiac disease. Being both conditions multifactorial, they share some genetic traits and pathogenic mechanisms, which are responsible for the typical skin and gastrointestinal manifestations. In dermatitis herpetiformis, skin and other lesions heal after gluten-free diet and reappear shortly after its reintroduction to complete diet. Prevalence of celiac disease is 1% in the population, and approximately 13% of patients with the disease develop dermatitis herpetiformis. Diagnosis of celiac disease has progressively increased in recent decades, while clinical manifestations become more and more diverse. Given the current high frequency of skin lesions in celiac patients, in this review we update relevant aspects of the epidemiology, pathogenesis, clinical presentations, treatment and follow up of dermatitis herpetiformis, as a contribution to improve the management of both conditions.

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RESUMEN Las enfermedades ampollares autoinmunes (EAA) comprenden un conjunto heterogéneo de patologías de baja frecuencia de presentación con evolución crónica, remisiones, exacerbaciones y una importante morbimortalidad aun con tratamiento temprano y adecuado. Existen pocos estudios epidemiológicos en nuestra población que incluyan todo el espectro de enfermedades ampollares. Objetivos : Determinar las características clínico-epidemiológicas de las EAA, describir tratamientos realizados, tiempo de control de actividad de la enfermedad y tiempo desde iniciado el tratamiento hasta la remisión completa. Determinar recaídas de las patologías estudiadas, evaluando si existe relación entre las mismas y algún factor reconocido. Materiales y métodos : Estudio retrospectivo y descriptivo. Se incluyeron todos los pacientes con diagnóstico clínico, histopatológico y de inmunofluorescencia de las EAA que hayan tenido seguimiento por al menos un año en el servicio de dermatología del HPUC, en el período de enero de 2008 hasta noviembre de 2018. Resultados : Se incluyeron 53 pacientes de los cuales el 50,9% tenía Penfigoide Ampollar (PA), 22,6% Dermatitis Herpetiforme (DH) y 17% de Pénfigo vulgar (PV). La edad de los pacientes se encontró entre 21 y 90 años. Las ampollas tensas y erosiones fueron las presentaciones más comunes. Los tratamientos sistémicos se realizaron en el 74% de los casos. Registramos recaídas en el 63% de los casos y dos casos de muerte durante el tratamiento. Se estableció más frecuencia de enfermedad cardiovascular en los pacientes con PA. Conclusiones : Es el primer trabajo retrospectivo sobre EAA en nuestro medio. Nuestros hallazgos permiten determinar una frecuencia importante de PA en la consulta dermatológica y de DH, hecho no reportado en otros estudios con poblaciones similares.

ABSTRACT The autoimmune bullous diseases (ASD) comprise a heterogeneous group of pathologies with a low frequency of presentation that presents a chronic evolution, with remissions, exacerbations and an important morbidity and mortality even with early and adequate treatment. There are few epidemiological studies in our population that include the entire spectrum of bullous diseases. Objectives : To determine the clinical-epidemiological characteristics of AAS, describe treatments performed, control time of disease activity and time from treatment initiation to complete remission. Determine relapses of the studied pathologies, evaluating if there is a relationship between them and some recognized factor. Materials and methods : Retrospective and descriptive study. All patients with clinical, histopathological and immunofluorescence diagnosis of the AAS who had followed up for at least one year in the dermatology service of the HPUC, from January 2008 to November 2018 were included. Results: 53 were included. patients of which 50.9% had Bullous Pemphigoid (BP), 22.6% Dermatitis herpetiformis (DH) and 17% Pemphigus Vulgaris (PV). The age of the patients was between 21 and 90 years. Tense blistering forms and erosions were the most common forms of presentation. Systemic treatments were performed in 74% of the cases. We recorded relapses in 63% of the cases and two cases of death during the treatment. More frequency of cardiovascular disease was established in patients with BP. Conclusions : It is the first retrospective work on EAA in our environment. Our findings allow determining an important frequency of PA in the dermatological consultation and DH, fact this last, not reported in other studies with similar populations.

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Abstract: Dermatitis herpetiformis and linear IgA bullous dermatosis are autoimmune diseases that present with pruritic urticarial papules and plaques, with formation of vesicles and blisters of subepidermal location, mediated by IgA antibodies. Mucosal lesions are present only in linear IgA bullous dermatosis. The elaboration of this consensus consisted of a brief presentation of the different aspects of these dermatoses and, above all, of an updated literature review on the various therapeutic options that were discussed and compared with the authors' experience, aiming at the treatment orientation of these diseases in Brazil. Dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and can be controlled with a gluten-free diet and dapsone. On the other hand, linear IgA bullous dermatosis arises spontaneously or is triggered by drugs, and can be controlled with dapsone, but often requires the association of systemic corticosteroids and eventually immunosuppressants.

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ABSTRACT BACKGROUND: Up to 15% of other immune-mediated diseases (IMDs) can occur in patients with CD throughout their lives and are associated with multiple factors, including sex and sex hormone levels. Moreover, sex is associated with differences in clinical presentation, onset, progression, and outcomes of disorders. OBJECTIVE: To investigate the prevalence of IMDs at diagnosis in patients with celiac disease (CD) and their first-degree relatives and to compare the findings between female and male patients of different age. METHODS: A retrospective study including Brazilian patients with CD who visited the same doctor during January 2012 to January 2017 was performed. Demographic and medical history data were collected through self-administered questionnaires and medical charts of the patients. In total, 213 patients were examined at diagnosis: 52 males (mean age, 40.0 years) and 161 females (mean age, 41.4 years). The patients were divided into two groups according to sex and age. RESULTS: IMDs were observed in 60.2% of the female (97/161) and 42.3% of the male patients (22/52; P=0.22). However, the frequency of IMDs was significantly higher in females aged 51-60 years than in males with same age (P=0.0002). Dermatitis herpetiformis (DH) was significantly more prevalent in males (P=0.02), whereas atopy was more prevalent in females (P=0.02). IMDs observed in first-degree relatives were similar to those observed in patients (70.9%; P<0.001), with a higher number observed in female relatives. CONCLUSION: The frequency of IMDs in CD patients was similar in all age groups and both sexes, except women diagnosed with CD after 51 years of age presented with an increased frequency of IMDs compared with males. Dermatitis herpetiformis was more prevalent in males, whereas atopy was more prevalent in females. No difference was observed in the type of IMDs between the first-degree relatives of both sexes.

RESUMO CONTEXTO: Até 15% das outras doenças imunomediadas (DIMs) podem ocorrer em pacientes com doença celíaca ao longo de suas vidas e estão associados a múltiplos fatores, incluindo sexo e níveis de hormônios sexuais. Além disso, o sexo está associado a diferenças na apresentação, início, progressão e desfecho das doenças. OBJETIVO: Investigar a prevalência de DIMs ao diagnóstico de doença celíaca e em seus familiares de primeiro grau e comparar os resultados entre sexo feminino e masculino em diferentes idades. MÉTODOS: Estudo retrospectivo incluindo pacientes brasileiros com diagnóstico de doença celíaca que realizaram acompanhamento com o mesmo médico no período de janeiro 2012 a janeiro de 2017. Dados demográficos e histórico médico foram coletados através de um questionário auto administrado e prontuários médicos dos pacientes envolvidos. No total, 213 pacientes eram portadores de doença celíaca, dos quais 52 do sexo masculino (idade média 40,0 anos) e 161 do sexo feminino (idade média 41,4 anos). Os pacientes foram divididos em dois grupos de acordo com o sexo e idade. RESULTADOS: DIMs foram observadas em 60,2% das pacientes femininas (97/161) e 42,4% dos pacientes masculinos (22/52; P=0,22). Entretanto, a frequência de DIMs foi significantemente maior em pacientes do sexo feminino com idade entre 51-60 anos que em pacientes masculinos da mesma idade (P=0,0002). Dermatite herpetiforme apresentou maior prevalência no sexo masculino (P=0,02), enquanto atopia obteve maior prevalência nas pacientes do sexo feminino (P=0,02). DIMs observadas em familiares de primeiro grau foram similares as encontradas nos pacientes (70,9%; P<0,001), com um maior número observado em familiares femininos. CONCLUSÃO: A frequência de DIMs em pacientes com doença celíaca foi similar nos grupos etários e ambos sexos, exceto as mulheres com diagnóstico de doença celíaca após a idade de 51 anos, as quais apresentaram um aumento na frequência de DIMs em comparação com os pacientes do sexo masculino. Dermatite herpetiforme apresentou maior prevalência em pacientes do sexo masculino, enquanto que atopia foi mais prevalente no sexo feminino. Em relação ao sexo, não foi observada diferença no tipo de DIMs observada entre os familiares de primeiro grau.

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RESUMEN: La Dermatitis Herpetiforme (DH) es una enfermedad ampollar autoinmune (EAI) que corresponde a la manifestación cutánea de la Enfermedad Celíaca (EC), más precisamente de la intolerancia al gluten. Clínicamente se manifiesta como una erupción papulo-vesicular pruriginosa, topografiada fundamentalmente en superficies de extensión de extremidades. El diagnóstico se realiza mediante el estudio histopatológico de piel lesional e inmunofluorescencia directa (IFD) de piel perilesional, la cual muestra hallazgos característicos. En su patogenia intervienen factores genéticos, inmunológicos, y ambientales. El tratamiento de elección es la dieta libre de gluten (DLG) y la Dapsona. Se ha reportado una asociación cercana al 15% entre la afectación cutánea e intestinal, no existe hasta el momento ningún estudio prospectivo que muestre la frecuencia real de EC en pacientes con DH

ABSTRACT: Dermatitis herpetiformis (DH) is an autoimmune blistering disease (EAI) that corresponds to the cutaneous manifestation of Celiac Disease (CD), more exactly of gluten intolerance. It is clinically manifested as an itchy papulovesicular rash, primarily located on extensor surfaces of the extremities. The diagnosis is made by histopathological examination of lesional skin and direct immunofluorescence (DIF) of surrounding skin, which shows characteristic findings. In its pathogenesis, genetic, immunologic and factors environmental factors take part. The treatment of choice is gluten-free diet (DEG) and dapsone. It has been reported an association of about 15% between the skin and intestinal involvement, there is so far no prospective study that shows the actual frequency of CD in patients with DH.