Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach

Abstract Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach.

Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

Gaze vaselinada para prevenção de lesões por pressão em pessoas com dermatoses imunobolhosas / Vaseline gauze for prevention of pressure injuries in people with immunobullous dermatoses / Gasa con vaselina para la prevención de lesiones por presión en personas con dermatosis inmunobulosa

Objetivo: avaliar a mobilidade do cliente com dermatose imunobolhosa antes e após aplicação do curativo com gaze vaselinada. Método: estudo quase experimental, interinstitucional, com clientes com dermatoses imunobolhosas hospitalizados em um hospital estadual e um hospital federal do Estado do Rio de Janeiro e uma instituição do Mato Grosso do Sul. Utilizou-se a lógica fuzzy para classificar a mobilidade dos sujeitos antes, 24 horas após e uma semana após aplicação do curativo. A pesquisa foi aprovada pelo Comitê de Ética em Pesquisa. Resultados: Incluídos 14 participantes, sendo nove com pênfigo vulgar, dois com pênfigo foliáceo e três com penfigóide bolhoso, entre 27 e 82 anos, predominando 11 mulheres. Após 24 horas, nenhum participante se considerou com baixa mobilidade, sete passaram a mobilidade média, e sete, alta, o que foi mantido uma semana após aplicação do curativo. Conclusão: constatou-se significativo aumento da mobilidade logo nas primeiras 24 horas após aplicação do curativo.

Objective: to assess the mobility of clients with immunobullous dermatoses, before and after applying vaseline gauze dressings. Method: in this quasi-experimental, interinstitutional study of inpatients with immunobullous dermatoses at a state hospital and a federal hospital in Rio de Janeiro State and an institution in Mato Grosso do Sul (Brazil), patient mobility before, 24 hours after, and one week after applying the dressing was classified using fuzzy logic. The study was approved by the research ethics committee. Results: 14 participants, nine with pemphigus vulgaris, two with pemphigus foliaceus, and three with bullous pemphigoid, aged between 27 and 82 years old, and predominantly (11) women. After 24 hours, none of the participants considered their mobility to be poor, seven began to be moderately mobile, and seven were highly mobile, and continued so one week after applying the dressing. Conclusion: mobility increased significant in the first 24 hours after applying the dressing.

Objetivo: evaluar la movilidad de clientes con dermatosis inmunobullosa, antes y después de la aplicación de apósitos de gasa con vaselina. Método: en este estudio cuasi-experimental, interinstitucional de pacientes hospitalizados con dermatosis inmunobullosa en un hospital estatal y un hospital federal en el estado de Río de Janeiro y una institución en Mato Grosso do Sul (Brazil), la movilidad del paciente antes, 24 horas después y una semana después la aplicación del apósito se clasificó mediante lógica difusa. El estudio fue aprobado por el comité de ética en investigación. Resultados: se incluyeron 14 participantes, nueve con pénfigo vulgar, dos con pénfigo foliáceo y tres con penfigoide ampolloso, con edades comprendidas entre 27 y 82 años, y predominantemente mujeres (n=11). Después de 24 horas, ninguno de los participantes consideró que su movilidad fuera pobre, siete comenzaron a ser moderadamente móviles y siete eran altamente móviles, y así continuaron una semana después de la aplicación del apósito. Conclusión: la movilidad aumentó significativamente en las primeras 24 horas después de la aplicación del apósitoconsideraba con baja movilidad, siete comenzaron a tener movilidad media y siete, alta, que se mantuvo una semana después de aplicar el apósito. Conclusión: hubo un aumento significativo en la movilidad en las primeras 24 horas después de aplicar el apósito.

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)

Assessment of the quality of life of Egyptian and Tunisian autoimmune bullous diseases' patients using an Arabic version of the autoimmune bullous disease quality of life and the treatment of autoimmune bullous disease quality of life questionnaires.

BACKGROUND: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. OBJECTIVES: We aimed to assess the ABQOL and TABQOL in the Arabic population. METHODS: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. RESULTS: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. STUDY LIMITATIONS: Small sample size of some AIBDs and patients with severe disease. CONCLUSION: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.

Assessment of the quality of life of Egyptian and Tunisian autoimmune bullous diseases' patients using an Arabic version of the autoimmune bullous disease quality of life and the treatment of autoimmune bullous disease quality of life questionnaires

Abstract: Background: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. Objectives: We aimed to assess the ABQOL and TABQOL in the Arabic population. Methods: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. Results: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. Study limitations: Small sample size of some AIBDs and patients with severe disease. Conclusion: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.

Immunoglobulin Therapy in a Patient With Severe Chikungunya Fever and Vesiculobullous Lesions.

Chikungunya virus (CHIKV) is an emerging arbovirus whose transmission has already been reported in several countries. Although the majority of individuals acutely infected with CHIKV appear to become asymptomatic, reports showing the occurrence of atypical and severe forms of the disease are increasing. Among them, the neurological and skin manifestations require medical attention. Treatment of CHIKV infection is almost symptomatic. In this sense, we report the case of a 56-years-old man who presented fever, headaches, paresthesia and pain in the right arm with visible red spots on the skin starting 30 days before Hospital admission. Tests determined Chikungunya infection and excluded other co-morbidities. Disease evolved with edema in hands and feet and extensive hemorrhagic bullous lesions on the skin of upper and lower limbs. Variations in hematological counts associated with liver dysfunction determined this patient's admission to the Intensive Care Unit. Then, he received intravenous antibiotic and immunoglobulin therapy (400 mg/Kg/day for the period of 5 days) with total recovery from the lesions after 10 days of follow-up. A general improvement in blood cell count and successful wound healing was observed. After discharge, no other clinical sign of the disease was reported until nowadays. This case reports for the first time the successful administration of intravenous immunoglobulin therapy to a patient with severe atypical dermatological form of Chikungunya Fever without any associated comorbidity.

Miíase associada a erisipela bolhosa / Case report: myiasis associated to bullous erysipelas

Miíase é a presença de larvas de moscas em tecidos do homem ou de outros animais vertebrados, onde se nutrem e evoluem como parasitos. Erisipela é uma celulite superficial que apresenta comprometimento do plexo linfático subjacente, cujo principal agente etiológico é Streptococcus (Rosenbach,1884) beta hemolítico do grupo A de Lancefield. Caracteriza-se por placas eritematosas acompanhadas de dor e edema. Este é o relato de um caso raro de paciente idosa internada em hospital público para tratamento de erisipela bolhosa no membro inferior esquerdo, em cujas lesões, durante a internação, foi detectada a presença de miíase. Foram retiradas várias larvas vivas com auxílio de pinça e prescrita ivermectina para erradicar possíveis larvas remanescentes. O diagnóstico precoce e o tratamento correto das lesões primárias são fundamentais para evitar a ocorrência de afecções como a miíase, cuja instalação atrasa o tratamento e pode agravar o prognóstico.

Myiasis is the presence of fly larvae in tissues of humans or other vertebrates,where they feed and develop as parasites. Erysipelas is a superficial cellulitis with involvement of underlying lymphatic plexus, characterized by erythematous plaques accompanied by pain and swelling. We report a case of an aged patient admitted to a public hospital for treatment of bullous erysipelas in the left lower limb, who developed cutaneous myiasis during hospitalization. Several larvae wereremoved with the aid of forceps and ivermectin was prescribed to eradicate possible remaining larvae. Early diagnosis and correct treatment of the lesions are essential to prevent the occurrence of diseases such as myiasis.

Neutrophilic dermatoses: part II.

This article addresses neutrophilic dermatoses, thus complementing the previous article (part I). The following dermatoses are introduced and discussed: subcorneal pustular dermatosis (Sneddon-Wilkinson disease), dermatitis cruris pustulosa et atrophicans, acute generalized exanthematous pustulosis, continuous Hallopeau acrodermatitis, palmoplantar pustulosis, infantile acropustulosis, Andrews' pustular bacteride and eosinophilic pustular folliculitis. A brief review of neutrophilic dermatoses in pediatric patients is also conducted.

Dermatoses neutrofílicas: parte II / Neutrophilic dermatoses: part II

Neste artigo são abordadas as dermatoses neutrofílicas, complementando o artigo anterior (parte I). São apresentadas e comentadas as seguintes dermatoses: pustulose subcórnea de Sneddon-Wilkinson, dermatite crural pustulosa e atrófica, pustulose exantemática generalizada aguda, acroder matite contínua de Hallopeau, pustulose palmoplantar, acropustulose infantil, bacteride pustular de Andrews e foliculite pustulosa eosinofílica. Uma breve revisão das dermatoses neutrofílicas em pacientes pediátricos também é realizada.

This article addresses neutrophilic dermatoses, thus complementing the previous article (part I). The following dermatoses are introduced and discussed: subcorneal pustular dermatosis (Sneddon-Wilkinson disease), dermatitis cruris pustulosa et atrophicans, acute generalized exanthematous pustulosis, continuous Hallopeau acrodermatitis, palmoplantar pustulosis, infantile acropustulosis, Andrews' pustular bacteride and eosinophilic pustular folliculitis. A brief review of neutrophilic dermatoses in pediatric patients is also conducted.

Autoimmune bullous dermatoses.

Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire), pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis.

Dermatoses bolhosas auto-imunes / Autoimmune bullous dermatoses

Dermatoses bolhosas autoimunes são doenças cuja manifestação cutânea primária e fundamental consiste em vesículas e bolhas. Classificam-se conforme a localização da bolha, em intraepidérmica e subepidérmica. Os pacientes produzem autoanticorpos contra estruturas específicas da pele detectáveis por técnicas de imunofluorescência, immunobloting e Elisa. Os recentes avanços da biologia molecular e celular têm permitido conhecer esses autoantígenos, contra os quais os pacientes se sensibilizam e que estão localizados na epiderme ou na junção dermoepidérmica. São doenças de baixa incidência, porém de elevada morbidade e por vezes letais. O objetivo deste trabalho é revisar e descrever os progressos nos conhecimentos de quatro doenças vésico-bolhosas autoimunes: pênfigo foliáceo endêmico (fogo selvagem), pênfigo vulgar, penfigóide bolhoso e dermatite herpetiforme.

Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire), pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis.

Bullous dermatoses in childhood: part II.

Bullous dermatoses are commonly encountered in childhood practice. Much confusion exists among clinicians because of the similarity of clinical lesions, paucity of relevant literature, and varied nomenclature used for these diseases. For a better understanding of the proposed classification, this second part is a review of diseases with nonhereditary characteristics of subepidermal cleavage and hereditary characteristics with intraepidermal and subepidermal cleavage.

Management of autoimmune bullous diseases: pharmacology and therapeutics

Bullous diseases are associated with high morbidity and mortality. They result from autoimmune response to one or more components of the basement membrane or desmosomes. Management consists of treating the immunologic basis of the disease, treating the inflammatory process involved in lesion formation, and providind supportive care both locally and systemically. Therapeutic agents are chosen based or their known pharmacologic properties and evidence of effectiveness derived from observations and studies

Alteraçöes dos tecidos moles da boca. Parte 1: doenças vesicobolhosas, condiçöes ulcerativas e lesöes verrucosas papilares / Alterations of bucal soft tissues. Part 1: vesicobullous diseases, ulcerative conditions and verrucous papilars lesions

Os autores iniciam o desenvolvimento do tema alteraçoes de tecidos moles da boca, esclarecendo à classe médica sobre as principais doenças vesicobolhosas, condiçoes ulcerativas e lesoes verrucosas papilares.

Pulsoterapia em buloses: relato de nove casos / Pulsotherapy in bullous disorders: report of nine cases

Os AA relatam os resultados obtidos com o uso intravenoso de doses suprafarmacológicas do succinato sódico de metilpredinisolona (1g/dia por três dias consecutivos) em nove casos e pentifigóide gestacional - um caso. A exceçäo de cinco pacientes virgens de tratamento, todos os demais apresentavam resistência ao tratamento oral com doses elevadas de corticosteróides ou alguma restriçäo ao seu uso prolongado. A pulsoterapia produziu remissäo completa das lesöes sem observaçäo de efeitos colaterais graves. O tratamento subseqüente consistiu do uso oral, de doses baixas de prednisona, isoladamente ou em associaçäo à azatioprina, ciclofosfamida, talidomida, ouro ou sufona. Durante o seguimento ambulatorial que variou de seis a 16 meses, foram registrados um abandono e duas recidivas

[Piezogenic nodules]. / Nódulos piezogénicos.

We make a brief bibliographic revision of the entity. We observe three cases with piezogenic pedal nodules, wrongly named painful piezogenic pedal papules; as they not always have that localization and symptomatology. An easy treatment is proposed, with an effective result to calm the pain when it appears.

Enfermedades vesiculo-ampollares / Vesicle-ampullar diseases

Fueron integradas bajo esta denominación un grupo de noxas que tienen en común la aparición de lesiones vesiculares y ampollares en forma crónica y recidivante. Hace una clasificacción de las enfermedades ampollares y refiere los aspectos etiológicos y terapéuticos de la dermatitis bullosas y el penfigo

Enfermedades ampollares crónicas no hereditarias en niños / Chronic non-hereditary blistering diseases in children

Se presenta una actualización sobre las dermatosis ampollares crónicas no hereditarias en niños. El diagnóstico diferencial entre éstas se basa en criterios clínicos, histopatológicos, ultramicroscópicos, inmunológicos e inmunogenéticos. Se comenta el tratamento recomendado para cada tipo. Se ilustra con fotografías de la clínica, la histopatología y la inmunofluorescenia directa de niños portadores de estas afecciones, atendidos en nuestro Hospital