A literature review and clinical consensus guidelines on the management of Bullous Pemphigoid

@#Bullous pemphigoid (BP) is the most common autoimmune blistering disease primarily characterized by tense blisters and occasionally with urticarial plaques, affecting the skin and mucous membranes. These are caused by autoantibodies against BP180 and BP230 which target antigens on the basement membrane zone. The diagnosis relies on the integration of clinical, histopathological, immunopathological, and serological findings. The management depends on the clinical extent and severity. We present in this article a literature review and the clinical consensus guidelines of the Immunodermatology Subspecialty Core Group of the Philippine Dermatological Society in the management of BP.

Childhood bullous pemphigoid: A case report

Introduction@#Bullous pemphigoid (BP) is an acquired autoimmune subepidermal bullous disease characterized by linear depo- sition of IgG and C3 along the basement membrane. It rarely occurs in childhood, especially in adolescence, with only 14 cases identified in literature. Treatment of choice is systemic corticosteroids but other treatment options such as anti-inflammatory antibacterials and methotrexate are available.@*Case report@#A 16-year-old Filipino girl presented with a three-month history of generalized vesicles and bullae. Nikolsky and Asboe-Hansen signs were negative. Histopathology and direct immunofluorescence were consistent with BP. ELISA to BP180 au- toantibody levels was elevated at 135 IU (normal <9 IU). Complete blood count showed leukocytosis with increase in neutrophils. Chest x-ray revealed pulmonary tuberculosis. The patient was given quadruple anti-Koch’s medication (pyrazinamide, rifampi- cin, ethambutol, isoniazid), prednisone, oral erythromycin and topical clobetasol propionate. Complete remission was attained at 10 months and is sustained at the time of writing.@*Conclusion@#To establish a definitive diagnosis and appropriate management, BP requires clinical, histopathologic, and immuno- logical correlation. Childhood BP has good prognosis and rapid treatment response, with rare relapses.

A case of anti-BP230 antibody-positive dyshidrosiform bullous pemphigoid secondary to dipeptidyl peptidase-4 inhibitor in a 65-year-old Filipino female

@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Bullous pemphigoid (BP) is a chronic, relapsing autoimmune blistering disorder commonly found in adults older than 60 years of age. It is mediated by autoantibodies directed against the hemidesmosomal proteins BP180 and BP230, which trigger an inflammatory cascade leading to blister formation. BP may present with pruritus, followed by an erythematous plaque or urticaria, and subsequently by bullae formation with or without mucosal involvement. It develops sporadically but can also be triggered by ultraviolet light exposure, radiation therapy, and medications such as dipeptidyl peptidase-4 inhibitor (DPP4i). Since 2006, the increasing use of DPP4i (also known as gliptins) for their good safety profi le in treating Type II Diabetes Mellitus has led to a further increase in the incidence of bullous pemphigoid.</p><p style="text-align: justify;"><strong>CASE REPORT:</strong> This is a case of a 65-year-old hypertensive and diabetic elderly Filipino female presenting DPP4i (linagliptin)-induced bullous pemphigoid with an atypical dyshidrosiform pattern, negative direct immunofluorescence (DIF), and Enzyme-linked immunosorbent assay (ELISA) that is negative for anti-BP180 antibodies but positive for anti-BP230 antibodies.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> The increasing use of DPP4i for diabetes mellitus for its good safety profile may be an essential contributing factor to the increasing incidence of BP in elderly hypertensive and diabetic patients with a simultaneous increasing incidence of atypical BP presentations such as the dyshidrosiform variant. Inability to recognize these factors carries significant therapeutic implications, including prolonged multidrug immunosuppression and increased patient morbidity and mortality.</p><p style="text-align: justify;"><strong>KEYWORDS:</strong> Bullous pemphigoid, gliptin, ELISA</p>

Penfigoide de las mucosas a lo largo de los años en nuestro Servicio / Mucous membrane pemphigoid through the years in our Department

Introducción: el penfigoide de las mucosas (PM), antes llamado mucoso, cicatrizal o mucosinequiante, representa un grupo heterogéneo de enfermedades ampollares autoinmunes inflamatorias crónicas que comprometen las mucosas o la piel, con tendencia a dejar secuelas cicatrizales. Existen autoanticuerpos contra distintos componentes de la zona de la membrana basal (BPAG1, BPAG2, integrina α6ß4, laminina 332, colágeno VII, entre otros), por lo que la inmunofluorescencia directa (IFD) es de suma importancia, así como la clínica, para su diagnóstico.Objetivo: realizar una revisión de los casos de PM diagnosticados durante un período de 24 años (enero de 1997- marzo de 2021) en el Sector de Enfermedades Ampollares del Hospital Ramos Mejía para determinar la epidemiología, la clínica y la terapéutica de esta enfermedad.Diseño: estudio retrospectivo descriptivo y observacional, en el que se analizaron las características clínicas e inmunopatológicas de 34 pacientes con diagnóstico de PM atendidos en el Servicio de Dermatología del Hospital Ramos Mejía desde enero de 1997 hasta marzo de 2021. Materiales y métodos: mediante las historias clínicas y los regis-tros iconográficos, se evaluaron las siguientes variables: prevalencia del diagnóstico de PM en los pacientes atendidos en el Sector, sexo, edad, antecedentes personales, mucosas afectadas, tiempo de evolución hasta el diagnóstico, hallazgos en la IFD, seguimiento clínico y tratamientos instaurados. Resultados: se estudió la evolución clínica de 34 pacientes diagnosticados con PM (5,3% del total de pacientes evaluados en el Sector de Patologías Ampollares). El sexo más afectado fue el femenino y la edad promedio en el momento del diagnóstico fue de 64 años. El 70,6% de los pacientes presentaron comorbilidades asociadas como hipertensión e hipotiroidismo. La mayoría refirió algún evento emocional como factor desencadenante. El sitio más comprometido fue la mucosa ocular y la cavidad oral fue la segunda en frecuencia. El tiempo de evolución promedio hasta el momento del diagnóstico fue de 4 años y 11 meses. El hallazgo más frecuente en la IFD fue la IgG lineal. El 17,6% de los pacientes interrumpieron el seguimiento clínico. El tratamiento más utilizado fue el mofetil micofenolato, con el que se obtuvo buena respuesta terapéutica. Conclusiones: el PM es una enfermedad autoinmune infrecuente que compromete las mucosas y, ocasionalmente, la piel. En este estudio, se observó que la principal mucosa afectada fue la conjuntival, a diferencia de lo referido en la bibliografía internacional dermatológica. El diagnóstico interdisciplinario temprano es fundamental para evitar las secuelas irreversibles.

Introduction: mucous membrane pemphigoid (MMP), also known as benign mucous membrane pemphigoid, cicatricial or mucosynechial pemphigoid, belongs to an heterogeneous group of chronic inflammatory autoimmune blistering diseases, which involves the mucous membranes (oral, ocular, pharyngeal, nasal, esophageal, laryngeal and anogenital) and/ or skin with tendency to scar formation. There are autoantibodies against different components of the basement membrane zone (BPAG 1- BPAG2, Integrin α6ß4, Laminin 332, Col VII, among others). The direct immunofluorescence (DIF) will be of paramount importance, as well as the clinical diagnosis.Objective: review the cases diagnosed with mucous membrane pemphigoid for 24 years (January 1997- March 2021) in the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital to establish the epidemiologic, clinic presentation and available treatments in this pathology.Design: retrospective descriptive and observational study of the clinical and immunopathological characteristics of 34 patients with MMP that were treated at the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital between January 1997 and March 2021.Materials and methods: though the medical histories and the photographic registries, we evaluated the following variables: prevalence of MMP within the patients that came to consult at the Blistering Disease Clinic at the Dermatology Department, sex, age, personal history, the affected mucous, evolution time until the diagnosis, direct immunofluorescence findings, clinical follow-ups and treatments.Results: we studied the clinical evolution of 34 patients diagnosed with MMP at our institution (5.3% from the total of patients at the Blistering Disease Clinic).The most affected gender was female and the average age at diagnosis was 64 years. 70.6% presented comorbidities such as hypertension and hypothyroidism. Most of our patients referred an emotional triggering event. The most affected membrane mucous was the ocular one and the oral was the second one. The delay in diagnosis was 4 years and 11 months. Linear deposits of IgG was the most frequent result in the direct immunofluorescence. 17.6% did not continue clinical follow-up. Mycophenolate mofetil was the most used drug with a good therapeutic response. Conclusions: MMP is a rare autoimmune disease that affects mucous membrane and occasionally the skin. In this study, the ocular involvement was the most frequent one, differing with the international reports. The early interdisciplinary diagnosis is essential to avoid irreversible sequelae.

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.

Penfigoide ampollar por teneligliptina / Teneligliptin-induced bullous pemphigoid

El Penfigoide Ampollar por fármacos es una variedad de penfigoide ampollar en la que un medicamento actúa como causa o desencadenante de la enfermedad. Clínicamente se manifiesta como ampollas tensas de contenido seroso localizadas fundamentalmente en abdomen, miembros superiores y raíz de muslos. El estudio histopatológico evidencia ampollas subepidérmicas e infiltrado dérmico mixto con eosinófilos. La inmunofluorescencia directa de piel sana perilesional muestra depósitos lineales de IgG y/o C3. Sin embargo, en hasta 15% de los casos puede ser negativa. Los pacientes diabéticos que reciben tratamiento con fármacos del grupo de los inhibidores de la dipeptidilpeptidasa 4, también conocidos como gliptinas, tienen 3 veces más riesgo de desarrollar esta patología. El tiempo de latencia entre el inicio de la medicación y la aparición de los síntomas es variable, con una media de 10 meses. El tratamiento radica en la suspensión inmediata del fármaco causal y la administración de prednisona oral 0,5 mg/kg/día. El tiempo medio de respuesta es de 10 días. Se presenta un varón de 82 años con una dermatosis ampollar pruriginosa de 3 semanas de evolución posterior al inicio de teneligliptina, cuyo estudio histopatológico fue característico de penfigoide ampollar, y que evolucionó satisfactoriamente al suspender el hipoglucemiante oral, sin aparición de nuevas lesiones a más de un año de seguimiento clínico

Drug-induced bullous pemphigoid is a variety of bullous pemphigoid in which a drug is the cause of the disease. It manifests as serous tense blisters located mainly on the abdomen, upper limbs and root of the tights. The histopathology shows subepidermal bullae and mixed dermal infiltrate with eosinophils. Direct immunofluorescence of healthy perilesional skin shows linear IgG and/or C3 deposits. However, it can be negative in up to 15% of the cases. Diabetic patients receiving dipeptidylpeptidase 4 inhibitors have a 3 times increased risk of developing drug-induced bullous pemphigoid. The mean time between the beginning of the medication and the appearance of the dermatosis is 10 months. Immediate suspension of the offending drug and administration of prednisone 0,5 mg/kg/day is the standard treatment. Average response time is 10 days. We present an 82-year-old-man with a 3-week itchy bullous dermatosis that started 8 months after treatment with teneligliptin, whose histopathological study resembled bullous pemphigoid, and which evolved satisfactorily when the drug was discontinued. No new lesions have been detected after more than one year of clinical follow-up. Key words: bullous pemphigoid, drug-induced bullous pemphigoid, gliptins, teneligliptin, dipeptidylpeptidase 4 inhibitors

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.

A case of bullous pemphigoid uniquely associated with dipeptidyl peptidase-4 (DPP-Inhibitor)

@#Introduction: Bullous pemphigoid (BP) is a chronic, autoimmune blistering disease occurring primarily in the elderly population. The pathogenesis of this condition has been strongly linked to the presence of circulating and tissue-bound autoantibodies against the basement membrane antigens BP180 and BP230. In most cases, the causative agent remains unidentified, but in a selected few, certain medications have been implicated in the pathogenesis of the disease. Dipeptidyl peptidase-4 inhibitors (-gliptins), in particular, which are used primarily in the treatment of diabetes mellitus, have been increasingly suspected to be a prime aggravating drug in the incidence of BP. Case summary: Bullous pemphigoid (BP) is a chronic autoimmune blistering disease mainly affecting the elderly population. While the pathogenesis has not yet been fully elucidated, it has been suggested that there is a correlation observed with certain groups of medications. Among drugs correlated with bullous pemphigoid, the group of dipeptidyl peptidase-4 inhibitors (-gliptins) used in the treatment of diabetes mellitus has been one of the most strongly associated. This is a case of a 64-year-old female on regular maintenance medications including linagliptin who developed generalized pruritus followed a week after by appearance of localized fluid-filled vesicles and bullae on the right lower leg. BP associated with dipeptidyl peptidase-4 inhibitors is characterized as “non-inflammatory” – lesions are localized and associated with less erythema compared to the classic presentation. Serum eosinophilia was absent, and serum autoantibody against BP180 was positive. Histopathologic and immunohistologic results revealed characteristics similar to classic bullous pemphigoid. The association of dipeptidyl peptidase-4 inhibitors to the development of BP was observed to have a long latency period between initiation of drug to onset of lesions. There was significant improvement after both withdrawal of the drug and standard steroids and doxycycline. Unlike other drug-induced BP, dipeptidyl peptidase-4 inhibitor-associated BP was found to have similar prognosis with the classic manifestation as the patient noted recurrence one month after remission despite withdrawal of inciting drug. Conclusion: There has been increasing incidence in DPP-4 inhibitor-associated BP. Though its clinical course is similar to classic BP, a non-inflammatory and more localized presentation would prompt suspicion of association with drug. The long latency in DPP-4 inhibitor and lesion onset suggests that rather than being simply an adverse reaction to treatment, DPP-4 inhibitor-associated BP should be viewed as a drug-associated or drug-aggravated disease. Determining the association of BP to DPP4-inhibitors is significant as the management for these patients not only entails standard management of BP but also withdrawal of the suspect drug, which in this case was found to significantly improve the patient’s lesions after one month. Unlike other drug-induced BP, however, DPP-4 inhibitor associated BP was found to have the same prognosis with classic BP as the patient noted recurrence one month after remission.

Clinical and Laboratory Features of 6 Cases of Pemphigoid Nodularis / 中国医学科学院学报

To investigate the clinical manifestations and laboratory characteristics of 6 cases of pemphigoid nodularis (PN). The clinical and laboratory data of 6 patients with PN admitted to the Department of Dermatology,Peking Union Medical College Hospital from January 2016 to August 2019 were retrospectively analyzed. PN mainly occurred in middle-aged and elderly people,with an average age of (58±16) years. Eosinophils were elevated in 4 patients. Immunoglobulin E (IgE) level was (530±672) kU/L in five patients. Direct immunofluorescence showed IgG and/or C3 deposition on basal membrane zone. Indirect immunofluorescence showed positive IgG anti-basement membrane zone,with a titer of 1:40-1:320. Enzyme-linked immunosorbent assay showed the anti-BP180 antibodies were positive [24-85 U/ml,average(43±26) U/ml] in 5 patients. None of the patients had neurological disorders. One patient was lost to follow-up. The disease recurred in 3 of 5 patients during the follow-up,and two patients still received maintenance corticosteroids. PN mainly occurs in middle-aged and elderly individuals. It is featured by elevated eosinophils and total IgE and relatively low anti-BP180 antibody titers. Recurrence is common but PN is less likely to be associated with neurological diseases.

Penfigoide gestacional y erupción ampollar en el recién nacido / Bullous pemphygoid and blisters in the newborn

El penfigoide gestacional es una dermatosis rara, que se presenta durante el embarazo. Se caracteriza por una respuesta autoinmune contra las proteínas de los hemidesmosomas, que genera un clivaje entre la epidermis y la dermis tanto de la piel como de las mucosas. Clínicamente, presenta prurito intenso, placas y pápulas eritematosas, que evolucionan a apollas con distribución en el abdomen y los miembros. Como complicaciones, en el feto puede generar parto prematuro y bajo peso para la edad gestacional, con alto riesgo de mortalidad. (AU)

Gestational pemphygoid is a rare, autoimmune dermatosis that occurs during pregnancy. It is characterized by an autoimmune response against hemidesmosome proteins, generating a cleavage between the epidermis and the dermis in the skin and mucous membranes. Clinically it presents with intense pruritus, plaques and erythematous papules that evolve to blisters that are distributed mainly in the abdomen and limbs. The complications are preterm birth and low weight for gestational age, with high risk of mortality. (AU)

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.

Bullous pemphigoid associated with psoriasis: a good response to methotrexate

Abstract Psoriasis has been associated with various autoimmune diseases, however, its relation to bullous diseases is infrequent. Of these, bullous pemphigoid appears as the main associated entity, even though both conditions differ considerably in demographic and clinical aspects. We report the case of a 42-year-old female patient, with long-standing psoriasis who consulted due to the exacerbation of psoriatic plaques associated with generalized bullous lesions on the skin and oral mucosa, with one-week duration. With clinical signs and histopathological findings compatible with bullous pemphigoid associated with psoriasis, we decided to treat her with methotrexate 10mg a week. The patient had an excellent response after two months of treatment.

Bullous pemphigoid

Abstract: Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.

Coagulation Disorders in Bullous Pemphigoid and Its Mechanism / 中国医学科学院学报

Bullous pemphigoid(BP)is an autoimmune blister disease caused by antibodies to the basement membrane zone.It usually exhibits tense bullae in skin and mucous membrane.BP patients can suffer from thrombosis due to hypercoagulation and/or hemorrhage due to the presence of anti-coagulation factors,which may be explained by the eosinophils,antibodies to the coagulation factors,and fibrinolytic system hyperactivity or inhibition.

Concurrent Bullous Pemphigoid and Pemphigus Vulgaris / 대한피부과학회지

No abstract available.

Bullous pemphigoid: can it occur in pediatric age? / Penfigoide ampolloso: ¿puede ocurrir en la edad pediátrica?

El penfigoide ampollar en niños es extremadamen-te infrecuente. La mayoría de los casos ocurre en adultos mayores, resultando fundamental conside-rar que en medicina todo es posible y esta enferme-dad igualmente podría presentarse en niños, por lo que se debe prestar especial atención a sus manifes-taciones clínicas, realizar los exámenes apropiados para descartar diagnósticos diferenciales y de esta forma, iniciar un tratamiento eficaz en el momen-to preciso. A continuación, presentamos un caso clínico de penfigoide ampollar que ocurrió en un paciente pediátrico atendido en nuestro hospital universitario.

Bullous pemphigoid in children is extremely in-frequent. Most of them predominate in elderly persons, resulting fundamental to consider that in medicine everything could be possible, and this disease could occur in children, so we must pay special attention to its clinical manifestations, to take the appropriate exams to rule out differen-tial diagnoses and in this way perform an effective treatment at the precise moment. Following, we present a clinical case of bullous pemphigoid that occurred in a pediatric patient attended at our university hospital.

Cuidados de enfermagem para conforto de pessoas com dermatoses imunobolhosas: avaliação pela lógica fuzzy / Nursing care to comfort people with immunobullous dermatoses: evaluation by fuzzy logic / Cuidados de enfermería para la comodidad de personas con dermatosis inmunoampollares: evaluación por la lógica fuzzy

Objetivos: avaliar se as intervenções propostas na tecnologia de cuidados de enfermagem ao cliente com dermatoses imunobolhosas contribuem para reduzir o desconforto, reconhecer padrões de desconforto antes e após aplicação da tecnologia. Método: aplicação do protocolo de avaliação em 14 clientes hospitalizados em enfermarias de dermatologia do Rio de Janeiro e Mato Grosso do Sul, Brasil. Os cuidados foram realizados mediante diagnósticos identificados e recomendações da tecnologia. A subjetividade em reco¬nhecer padrões de conforto em clientes com doenças raras direcionou o uso da lógica fuzzy em função dos atributos dor, mobilidade, padrão de sono, exposição do corpo/lesões. Aprovado conforme CAAE: 0258.0.228.000-11. Resultados: dos 14 participantes, oito verbalizaram redução da dor após 24 horas. Após uma semana, três declararam maior redução, cinco manutenção e cinco aumento. Conclusão: a análise inferencial fuzzy propiciou avaliar padrões de desconforto, apontando para a veracidade da hipótese de que a tecnologia contribui para promover o conforto da clientela.

Objectives: to evaluate whether interventions proposed in nursing care technology for clients with immunobullous dermatoses contribute to reducing discomfort, and to recognize patterns of discomfort before and after application of the technology. Method: the evaluation protocol was applied to 14 patients hospitalized in dermatology wards in Rio de Janeiro and Mato Grosso do Sul, Brazil, from June 2012 to April 2013. Care was performed by way of identified diagnoses and recommendations in the technology. The role of subjectivity in rec¬ognizing comfort patterns in clients with rare diseases indicated the use of fuzzy logic with attributes of pain, mobility, sleep pattern, and body exposure/lesions. The study was approved under CAAE: 0258.0.228.000-11. Results: of the 14 patients, eight reported diminished pain after 24 hours. After one week, three reported larger reductions; five, no change; and five, increased pain. Conclusion: evaluation of patterns of discomfort by fuzzy inferential analysis supported the hypothesis that the technology contributes to promoting client comfort.

Objetivos: evaluar si las intervenciones propuestas en la tecnología de cuidados de enfermería al cliente con dermatosis inmunoampollares contribuyen a reducir el incómodo; reconocer patrones de incomodidad antes y después de la aplicación de la tecnología. Método: aplica¬ción del protocolo de evaluación en 14 pacientes hospitalizados en enfermerías de dermatología de Río de Janeiro y Mato Grosso do Sul, Brasil, de junio/2012 a abril/2013. Los cuidados se han realizado mediante diagnósticos identificados y recomendaciones de la tecnología. La subjetividad en reconocer patrones de comodidad en pacientes con enfermedades raras dirigió el uso de la lógica fuzzy en función de los atributos dolor, movilidad, patrón de sueño, exposición del cuerpo / lesiones. Aprobado conforme CAAE: 0258.0.228.000-11. Resultados: de los 14, ocho reportaron reducción del dolor después de 24 horas. Tras una semana, tres declararon una mayor reducción, cinco mantu¬vieron y cinco aumentaron. Conclusión: el análisis inferencial fuzzy propició evaluar patrones de incomodidad, señalando la veracidad de la hipótesis de que la tecnología contribuye a promover la comodidad de los pacientes.

Bullous pemphigoid: a series of 50 cases / Penfigoide bolhoso: série de 50 casos

OBJECTIVES: This study aims to analyze data on the epidemiology, treatment and course of bullous pemphigoid in 50 patients and compare findings to the data already available in the literature. METHODS: Data were collected retrospectively through medical records and analyzed statistically. A review of the literature was conducted using articles indexed in the MEDLINE (via PubMed) database. RESULTS: The mean age at diagnosis was 71.1 years. Comorbidities were observed in almost all cases, and the association between bullous pemphigoid and neurological diseases was present in 18% of patients, in agreement with recent data in the literature. CONCLUSION: Care of comorbidities, especially neurological diseases, which increase the mortality of patients with bullous pemphigoid, is thus essential

OBJETIVOS: Este estudo tem por objetivo analisar dados epidemiológicos, de tratamento e evolução de 50 pacientes com diagnóstico de penfigoide bolhoso e comparar aos dados já existentes na literatura. MÉTODOS: Os dados foram coletados retrospectivamente por meio de prontuários médicos e analisados estatisticamente. Foi realizada revisão da literatura mediante artigos indexados na base de dados MEDLINE (via PubMed). RESULTADOS: A média de idade ao diagnóstico foi de 71,1 anos. Comorbidades foram observadas em quase a totalidade dos casos, e a associação entre penfigoide bolhoso e doenças neurológicas esteve presente em 18% dos pacientes, em concordância com dados recentes da literatura. CONCLUSÃO: Ressaltamos a atenção às comorbidades, sobretudo doenças neurológicas, que aumentam a mortalidade dos pacientes com penfigóide bolhoso