Piel y tubo digestivo: dermatitis herpetiforme y enfermedad celíaca / Small bowel and skin: celiac disease and dermatitis herpetiformis

La dermatitis herpetiforme, también denominada Enfermedad de Dühring-Brocq, es una dermatosis autoinmune crónica que evoluciona por brotes, caracterizada por la presencia de ampollas pequeñas que tienden a agruparse, en codos, rodillas y glúteos, con disposición simétrica, intensamente pruriginosas. Es considerada una manifestación cutánea de la enfermedad celíaca. Afecta a adultos jóvenes (20 a 50 años). El estudio histopatológico evidencia ampollas subepidérmicas. La inmunofluorescencia directa es característica: depósitos granulares de IgA en las puntas de las papilas dérmicas. Aún ante falta de sintomatología digestiva debe investigarse enfermedad celíaca en todos los pacientes. La dieta libre de gluten es la clave del tratamiento. En aquellos pacientes con intenso prurito o con una dermatosis muy extensa se puede utilizar dapsona vía oral, que alivia rápidamente las manifestaciones cutáneas, pero no modifica el curso de la enfermedad digestiva. Se presenta un paciente en quien a partir de las lesiones cutáneas se realizó diagnóstico de dermatitis herpetiforme primero y de enfermedad celíaca luego

Dermatitis herpetiformis, also known as Dühring-Brocq disease, is a chronic autoimmune dermatosis that evolves in outbreaks. It is characterized by the presence of small blisters that tend to cluster on the elbows, knees, and buttocks, with a symmetrical distribution and intense itching. It is considered a cutaneous manifestation of celiac disease. It affects young adults (20 to 50 years old). Histopathological examination reveals subepidermal blisters. Direct immunofluorescence is characteristic, showing granular deposits of IgA at the tips of the dermal papillae. Even in the absence of digestive symptoms, celiac disease should be investigated in all patients. A gluten-free diet is the key to treatment. In patients with intense itching or extensive dermatosis, oral dapsone can be used to quickly relieve cutaneous manifestations, but it does not alter the course of the digestive disease. We present a patient in whom the diagnosis of dermatitis herpetiformis was made initially, followed by a diagnosis of celiac disease based on the skin lesions

Dermatitis herpetiforme como manifestación extraintestinal gluten inducida en la enfermedad celíaca: estudio retrospectivo de pacientes que se asisten en el Hospital de Clínicas Dr. Manuel Quintela / Dermatitis herpetiformis as a gluten induced extraintestinal manifestation in coeliac disease: retrospective study of patients assisted at "Dr. Manuel Quintela" University Hospital / Dermatite herpetiforme como manifestação extraintestinal induzida por glúten na doença celíaca: estudo retrospectivo de pacientes atendidos no Hospital de Clínicas "Dr. Manuel Quintela"

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.

Dermatitis herpetiforme y enfermedad celíaca: del intestino a la piel / Dermatitis herpetiformis and celiac disease

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.

Afecciones perianales no infecciosas, no neoplásicas, del adulto / Noninfectious, non-neoplasic adult perianal affections

Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)

Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)

Consensus on the treatment of autoimmune bullous dermatoses: dermatitis herpetiformis and linear IgA bullous dermatosis - Brazilian Society of Dermatology

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.

The importance of direct immunofluorescence in pemphigus herpetiformis diagnosis

Abstract Pemphigus herpetiformis is an autoimmune bullous disease, that combines clinical features of dermatitis herpetiformis and linear IgA bullous dermatosis and immunological characteristics of pemphigus, which makes this disease peculiar and this diagnosis rarely suspected in the first evaluation of the patient. The reported case is of a patient with clinically bullous disease similar to dermatitis herpetiformis, whose multiple biopsies were inconclusive, and only after direct immunofluorescence with a pemphigus pattern (intraepidermal intercellular pattern) the confirmation of the diagnosis was possible.

Dermatitis herpetiforme y enfermedad celíaca / Dermatitis herpetiformis and celiac disease

La dermatitis herpetiforme (DH) es una enfermedad crónica y ampollar caracterizada por la presencia de lesiones intensamente pruriginosas, de ubicación característica, y por asociarse en todos los casos a enfermedad celíaca (EC) (sintomática o no). Ambas entidades se consideran una expresión, en diferentes órganos, de hipersensibilidad al gluten. Se presenta una serie de cuatro pacientes de sexo femenino, con un promedio de 46 años, que consultaron por la aparición de pápulas, lesiones erosivocostrosas, excoriaciones y ampollas, pruriginosas, localizadas predominantemente en los codos, las rodillas y el dorso superior. Referían brotes intermitentes con un tiempo de evolución de entre 6 meses y 10 años. Se realizó una biopsia cutánea y estudio histopatológico que evidenció la presencia de una dermatosis ampollar subepidérmica con neutrófilos e IFD positiva en tres de las pacientes, y que confirmó el diagnóstico de dermatitis herpetiforme. Los hallazgos de laboratorio y la videoendoscopia digestiva alta con toma de biopsia fueron compatibles, en todos los casos, con enfermedad celíaca. Se les indicó dieta libre de gluten (DLG) a todas las pacientes; en una de ellas fue suficiente para lograr la remisión completa de las lesiones después de 3 meses; las tres restantes requirieron tratamiento con dapsona para controlar la enfermedad (AU)

Dermatitis herpetiformis (DH) is a chronic, bullous disease, which is characterized by intensely pruritic lesions, property location and diagnosis in all cases of celiac disease (CD) (symptomatic or not). Both entities are considered expression in different organs of hypersensitivity to gluten. A series of four female patients is presented with an average of 46 years who consulted by the appearance of papules, erosivocostrosas injuries, abrasions and blisters, itchy, localized predominantly on elbows, knees and upper back. Intermittent outbreaks concerned with evolution time between 6 months and 10 years. IFD positive skin biopsy and histopathological study showed subepidermal bullous dermatosis with neutrophils was performed, and in three of the patients confirmed the diagnosis of dermatitis herpetiformis. Laboratory findings and upper gastrointestinal video endoscopy with biopsy were compatible in every case with celiac disease. Gluten-free diet in all patients indicated, one of them was enough to achieve complete remission of lesions after three months; the remaining three required starting dapsone for disease control (AU)

Dermatosis ampollar por inmunoglobulina A lineal: Reporte de dos casos / Linear immunoglobulin A bullous dermatosis: Two cases report

La dermatosis ampollar por inmunoglobulina A lineal es una rara enfermedad, generalmente autolimitada, que afecta a niños de 4,5 años (edad media), con una incidencia de 0,52,3 casos/millón de habitantes/año. Es, tras la dermatitis herpetiforme, la enfermedad ampollar pediátrica más frecuente. Ocurre en brotes con lesión patognomónica en collar de perlas y afecta preferentemente la zona genital y peribucal. Su diagnóstico se basa en una alta sospecha clínica y en la biopsia de piel con observación de ampollas subepidérmicas y depósito lineal de inmunoglobulina A en inmunofluorescencia directa. Frecuentemente, el diagnóstico es tardío debido al desconocimiento de esta enfermedad.

Linear immunoglobulin A bullous dermatosis is a rare entity with frequent spontaneous resolution. It usually presents in children with average age of 4.5 years. Its incidence is about 0.5-2.3 cases/million individuals/year. It is, after dermatitis herpetiformis, the most frequent paediatric blister disorder. It usually appears in bouts with acute development of vesicles in strings of pearls; affecting the perioral area and genitalia. Diagnosis is based on the clinical signs and symptoms and biopsy of the skin with subepidermal blister and a linear band of immunoglobulin A in the direct immunofluorescence. Often, diagnosis is made late because of the unawareness of this disease.

Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment

Researches on DH have shown that it is not just a bullous skin disease, but a cutaneous-intestinal disorder caused by hypersensitivity to gluten. Exposure to gluten is the starting point of an inflammatory cascade capable of forming autoantibodies that are brought to the skin, where they are deposited, culminating in the formation of skin lesions. These lesions are vesico-bullous, pruritic, and localized especially on elbows, knees and buttocks, although atypical presentations can occur. Immunofluorescence of perilesional area is considered the gold standard for diagnosis, but serological tests help in cases where it is negative. Patients who follow glutenfree diets have better control of symptoms on the skin and intestine, as well as lower risks of progression to lymphoma. Dapsone remains the main drug for treatment, but it requires monitoring of possible side effects, some potentially lethal.

Detección de anticuerpos circulantes en biopsia de tejido para el diagnóstico de dermatitis herpetiforme / Detection of circulating antibodies in tissue biopsy for the diagnosis of dermatitis herpetiformis

Introducción: la dermatitis herpertiforme (DH), es una enfermedad crónica inflamatoria de la piel. Se caracteriza por la presencia de lesiones pruriginosas de diversas formas y de hallazgos histopatológicos típicos. Se ha considerado como la expresión cutánea de la enteropatía sensible al gluten (ESG), indistinguible de la enfermedad celiaca. Es una entidad con baja prevalencia y se presenta con más frecuencia en la tercera década de la vida. Afecta principalmente a individuos caucásicos. El diagnóstico de realiza a través de la detección de un depósito granular de Inmunoglobulina A (IgA) en la unión dermoepidérmica, a través de un estudio de inmunofluorescencia directa (IFD) de una muestra de piel sana perilesional. Estos depósitos con frecuencia se localizan en las puntas de las papilas dérmicas. La negatividad del estudio de inmunofluorescencia directa debe hacer dudar del diagnóstico. El tratamiento consiste en mantener una dieta estricta libre de gluten, el manejo de las lesiones y la prescripción de dapsona para el manejo de las erupciones cutáneas. Objetivo: evaluar la utilidad de la detección de anticuerpos circulantes en biopsia de tejido para el diagnóstico de la dermatitis herpetiforme. \r\nResultados: se identificaron 174 publicaciones. Con los resultados obtenidos, no fue posible identificar revisiones sistemáticas de la literatura ni estudios de validez diagnóstica de la IFD. Se hizo una preselección de 18 estudios observacionales descriptivos. Fueron incluidos nueve series de casos. Se presentan los datos descriptivos sobre la positividad de la IFD para el diagnóstico de la DH. Conclusiones: \r\nactualmente se considera que la IFD es el patrón de oro para el diagnóstico de la DH.(AU)

Pustular psoriasis of pregnancy (Impetigo herpetiformis) - case report / Psoriase pustulosa da gestacao (Impetigo herpetiforme) - relato de caso

Impetigo herpetiformis is a rare dermatosis of pregnancy with typical onset during the last trimester of pregnancy and rapid resolution in the postpartum period. Clinically and histologically, it is consistent with pustular psoriasis. This similarity has led some authors to name the disease "the pustular psoriasis of pregnancy". We report the case of a patient who developed impetigo herpetiformis in two sucessive pregnancies.

Impetigo herpetiforme é uma dermatose gestacional rara que se inicia tipicamente durante o último trimestre e evolui com rápida resolução no período pós-parto. Clinica e histologicamente é consistente com psoríase pustulosa. Essa similaridade tem levado alguns autores a nomearem a doença como "psoríase pustulosa da gestação". Relatamos o caso de uma paciente que apresentou impetigo herpetiforme em duas gestações subsequentes.

Dermatitis herpetiformis as the only manifestation of celiac disease: case report and literature review / Dermatite herpetiforme como única manifestação de doença celíaca: relato de caso e revisão da literatura

Aims: To present a case of dermatitis herpetiformis, a papulovesicular rash due to deposits of immunoglobulin A in the papillary dermis. This is a common extraintestinal manifestation of celiac disease, although rare in childhood.Case description: A 10-year-old girl was diagnosed with celiac disease, suspected only due to the occurrence of typical lesions of dermatitis herpetiformis. Intestinal biopsy demonstrated total atrophy of duodenal villi in spite of the lack of clinical digestive manifestations. Under a gluten-free diet the patient presented favorable evolution, with regression of cutaneous lesions.Conclusions: Dermatitis herpetiformis is a common manifestation of celiac disease, but is not frequent in infants. Therefore, is very important to investigate any child that presents a chronic papulovesicular cutaneous eruption non-responsive to usual treatments in order to perform a precocious diagnosis of celiac disease, avoiding its serious repercussions...

Objetivos: Apresentar um caso de dermatite herpetiforme, uma erupção cutânea papulovesicular pruriginosa devida ao depósito de imunoglobulina A na derme papilar. Esta é uma manifestação extraintestinal comum da doença celíaca, embora rara na infância.Relato de caso: Uma paciente com 10 anos de idade foi diagnosticada com doença celíaca, cuja suspeita surgiu unicamente em decorrência de lesões típicas de dermatite herpetiforme. A biópsia intestinal demonstrou atrofia total das vilosidades duodenais, apesar da ausência de manifestações clínicas digestivas. Com dieta livre de glúten a paciente apresentou evolução favorável, com regressão das lesões cutâneas.Conclusões: A dermatite herpetiforme é uma manifestação comum da doença celíaca que, no entanto, é infrequente na infância. Por isso, é fundamental alto grau de suspeição em qualquer criança que apresentar uma erupção cutânea papulovesicular crônica não responsiva a medidas simples, a fim de realizar o diagnóstico precoce da doença celíaca, evitando suas graves repercussões...

Dermoscopy examination of petechial lesions in a patient with Dermatitis Herpetiformis / Exame dermatoscopico de lesoes petequiais num paciente com Dermatite Herpetiforme

An uncommon skin manifestation of Dermatitis Herpetiformis is palmar and plantar purpura. Dermoscopic examination is useful for any skin condition since it allows recognition of structures that are not discernible to the naked eye. A 22 year-old Caucasian man was admitted with excoriated lesions and pruritus. Petechial lesions could be seen on volar aspect of the digits on the hands and feet. Dermoscopy examination revealed erythematous and violaceous dots and erythematous and brown dots.

Púrpura palmar e plantar é uma manifestação cutânea incomum da Dermatite Herpetiforme. A dermatoscopia é útil para o exame dermatológico por permitir o reconhecimento de estruturas que ao olho nu não são perceptíveis. Doente caucasiano masculino de 22 anos que queixava-se de lesões escoriadas e prurido. Observouse na face volar dos dedos dos pés e das mãos lesões petequiais. O exame dermatoscópico revelou pontos eritematosos e violáceos, além de pontos eritematosos e marrons.

Manifestaciones cutáneas de enfermedades sistémicas / Cutaneous manifestations of systematic diseases

Un gran número de enfermedades sistémicas durante su evolución pueden presentar manifestaciones cutáneas, enocasiones propias de la dolencia, aunque en otras pueden ser el único indicador de la presencia de una enfermedadinterna asintomática, por ello es fundamental su reconocimiento. Su aparición debe alertar al médico sobre el probableinicio de ciertos desórdenes sistémicos, lo que permitirá realizar un diagnóstico precoz, con las implicanciasclínicas y terapéuticas de intervención a corto y largo plazo. El propósito de este trabajo es describir las lesionescutáneas que con mayor frecuencia se asocian a enfermedades internas, lo cual puede ser de gran utilidad para elejercicio de médicos generalistas, internistas y dermatólogos.

Skin signs of systemic diseases occur frequently, and sometimes constitute the first symptoms of an internal disease; furthermore,these manifestations may be the sole expressions of otherwise asymptomatic systemic disorders, so it is neecessary tobecome acquainted with them. Their appearance should alert us of the likely onset of a systemic disorder, which allows anearly diagnosis, with clinical and therapeutic implications both in the short and the long term. We describe the clinicalfeatures of skin lesions observed in several internal diseases which will be useful to general practitioners, internists anddermatologists in the diagnosis of systemic diseases.

Review: dermatitis herpetiformis / Revisao: dermatite herpetiforme

Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules and urticarial plaques, grouped vesicles with centrifuge growth, and tense blisters. There is an association with the genotypes HLA DR3, HLA DQw2, found in 80-90% of cases. It is an IgA-mediated cutaneous disease, with immunoglobulin A deposits appearing in a granular pattern at the top of the dermal papilla in the sublamina densa area of the basement membrane, which is present both in affected skin and healthy skin. The same protein IgA1 with J chain is found in the small intestinal mucosa in patients with adult celiac disease, suggesting a strong association with DH. Specific antibodies such as antiendomysium, antireticulina, antigliadin and, recently identified, the epidermal and tissue transglutaminase subtypes, as well as increased zonulin production, are common to both conditions, along with gluten-sensitive enteropathy and DH. Autoimmune diseases present higher levels of prevalence, such as thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%) and collagen tissue disease. The chosen treatment is dapsone and a gluten-free diet.

Dermatite herpetiforme é uma doença bolhosa crônica caracterizada por intenso prurido e sensação de queimação em pápulas eritematosas e placas urticariformes, vesículas agrupadas com crescimento centrífugo e bolhas tensas. Apresenta associação com genótipos de HLA DR3, HLA DQW2 encontrados em 80 a 90% dos casos. É uma doença cutânea mediada por IgA com depósito de imunoglobulina A em padrão granular no topo da papila dérmica na área da sublâmina densa na zona da membrana basal, presente tanto na pele lesada com em área de pele sã. A mesma cadeia J da proteína IgA1 é encontrada na mucosa do intestino delgado em pacientes com doença celíaca do adulto, sugerindo forte associação com a dermatite herpetiforme. Anticorpos específicos com anti-endomísio, anti-reculina, anti-gliadina, e recentemente identificado, o subtipo transglutaminase epidérmica e tecidual, assim como a produção aumentada da zonulina, são descritas em ambas as afecções enteropatia sensível ao glúten e a deramtite herpetiforme. Exibe depósitos de IgA em padrão granular na papila dérmica. Doenças auto-imunes exibem maior prevalência como tireoidopatia em 5 a 11%, anemia perniciosa em 1 a 3%, diabetes tipo 1 em 1 a 2% e doença do colágeno. O tratamento de escolha é a dapsona e dieta isenta de glútem.

Case for diagnosis / Caso para diagnóstico

We report a clinical case of a rare variant of pemphigus - pemphigus herpetiformis - which combines the clinical features of dermatitis herpetiformis with the immunological findings of pemphigus. Due to its atypical presentation, it is frequently misdiagnosed as dermatitis herpetiformis. It is basically characterized by the herpetiform pattern of skin lesions, severe pruritus and by the presence of eosinophilic spongiosis confirmed on histopathology. We call attention to the excellent response to dapsone.

Descrevemos um caso clínico de uma variante rara de pênfigo - pênfigo herpetiforme - que combina os aspectos clínicos da dermatite herpetiforme com os achados imunológicos do pênfigo. Devido à sua apresentação atípica, é frequentemente diagnosticado equivocamente como dermatite herpetiforme. Caracteriza-se essencialmente pelo padrão herpetiforme das lesões cutâneas, prurido intenso e presença de espongiose eosinofílica no exame histopatológico. Enfatizamos a excelente resposta terapêutica à dapsona.

Lúpus eritematoso sistêmico bolhoso: diagnóstico diferencial com dermatite herpetiforme / Bullous systemic lupus erythematosus: differential diagnosis with dermatitis herpetiformis

O lúpus eritematoso sistêmico bolhoso é um subtipo raro do lúpus eritematoso sistêmico, que ocorre ainda de forma mais incomum nos pacientes pediátricos. Relatamos o caso de uma adolescente de 12 anos, apresentando lesões vésico-bolhosas em face, pescoço, tronco, mucosas oral e genital, anemia, leucocitúria estéril, FAN: 1/1280 padrão nuclear pontilhado grosso, Anti-Sm e Anti-RNP positivos. O estudo anatomopatológico sugere dermatite herpetiforme e a imunofluorescência direta revela IgG, IgA e fibrina ao longo da zona de membrana basal. Apresentamos um caso típico de lúpus eritematoso sistêmico bolhoso e enfatizamos a importância do diagnóstico diferencial com a dermatite herpetiforme.

Bullous systemic lupus erythematosus is a rare subset of systemic lupus erythematosus that is even rarer in pediatric patients. We report a case of a 12-year-old girl who presented with a vesiculobullous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP. Pathological examination suggested dermatitis herpetiformis, and direct immunofluorescence revealed IgG, IgA and fibrin in the epithelial basement membrane zone. We present a typical case of bullous systemic lupus erythematosus and emphasize the importance of clinical and histopathological differential diagnosis with dermatitis herpetiformis.