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.

Penfigoide buloso nodular tratado exitosamente con fototerapia UVB banda angosta / Pemphigoid nodularis successfully treated with narrow band UVB phototherapy

El Penfigoide nodular es una variante clínica poco frecuente de penfigoide buloso. Corresponde a una dermatosis ampollar subepidérmica, crónica, autoinmune, caracterizada por auto anticuerpos contra antígenos específicos de hemidesmosomas en la unión dermo-epidérmica. Su incidencia es desconocida. La etiopatogenia aún no es entendida del todo. Se presenta clínicamente como una superposición de características de pénfigo buloso y prurigo nodular. El diagnóstico se basa en hallazgos clínicos e inmunopatológicos. La histopatología con inmunofluorescencia directa es el gold standard para el diagnóstico. El manejo es difícil, tiene mala respuesta a corticoides potentes locales, siendo necesario el uso de corticoides sistémicos y diferentes inmunosupresores solos o combinados junto a antihistamínicos para el manejo de prurito intenso. Se presenta un caso de pénfigo nodular, donde destaca su buena respuesta a terapia combinada con metotrexato y luz UVB de banda angosta.

Pemphigoid Nodularis is a rare clinical variant of bullous pemphigoid. It is considered an autoimmune, chronic, subepidermal blistering dermatosis, characterized by antibodies against hemidesmosome-specific antigens at the dermo-epidermal junction. Its incidence is unknown and its etiopathogenetic not fully understood. Clinically, it presents with overlapping features of bullous pemphigoid and prurigo nodularis. The diagnosis is based on clinical and immunopathological findings, being the histopathological study with immunofluorescence the gold standard. The management is difficult; since it has a poor response to local potent corticosteroids, requiring the use of systemic corticosteroids and different immunosuppressants alone or combined with antihistamines for the intense pruritus. We present a case of nodularis pemphigoid, highlighting the good response to the combination of methotrexate and phototherapy with narrow band UVB.

Penfigoide ampolloso / Bullous pemphigoid

El penfigoide ampolloso (PA) es una enfermedad cutánea ampollar subepidérmica autoinmunitaria que habitualmente se presenta en ancianos. Clásicamente el PA se caracteriza por presentar ampollas tensas, grandes en piel normal o eritematosa, con o sin prurito. Es difícil identificar al penfigoide ampollar prodrómico (PAP) o en estadio temprano. El PAP no se presenta con lesiones vesiculares o ampollar es como el penfigoide clásico. Se han publicado reportes en los que la enfermedad se manifiesta como prurito generalizado sin lesiones visibles, placas urticarianas, placas eritematosas, parches de eccema y dishidrosis, lesiones tipo prúrigo nodular, eritrodermia y lesiones vegetantes. Se reporta el caso de un paciente en la octava década de su vida que acude a nuestro servicio por presentar cuadro clínico e histopatológico de PA; mediante la anamnesis encontramos que debutó con la forma no ampollar del penfigoide, siendo tratado por diferentes diagnósticos sin mejoría hasta que presentó la fase ampollar clásica siendo el diagnóstico evidente. Presentamos este caso para tener en cuenta dentro del diagnóstico diferencialde aquellas lesiones inespecífica de curso tórpido, el estadio temprano del PA, pues un reconocimiento y tratamiento y temprano puede prevenir la progresión a PA clásico cuyo curso es crónico, con fases de exacerbaciones y remisiones espontáneas, que puede resultar en morbilidad significativa.

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease that typically occurs in the elderly. Classically (BP) is characterized by tense blisters, large normal or erythematous skin, with or without pruritus. Itis difficult to identify the prodromal bullous pemphigoid(PBP) or early stage. The PBP is not presented with vesicular lesions or bullous pemphigoid such as classic. Reports have been published in which the disease manifests as generalized pruritus without visible lesions, urticarial plaques, erythematous plaques, patches of eczema and dyshidrosis, nodular prurigo-like lesions, erythroderma and vegetative lesions. We report the case of a patient inthe eighth decade of his life referred to our department to present clinical and histopathological picture bullouspemphigoid; through history we find that debuted with the bullous pemphigoid is not being treated for different diagnoses did not improve until presented by the classic blister stage diagnosis being evident. We present this case to account for the differential diagnosis of those injuries nonspecific chronic course, the early stage of the BP, asan early recognition and treatment can prevent progression to classical PA whose course is chronic, with periods of exacerbations and spontaneous remissions, which can result in significant morbidity.

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.

Niño de cuatro meses de vida con lesiones ampollares / Child of four months with bullous lesions

Presentamos un niño de 4 meses de edad con un cuadro clínico típico de PA, con las ampollas características y en localizaciones habituales en menores de un año, (predominio en palmas y plantas y respeto de la zona del pañal), con leucocitosis y eosinofilia en los exámenes de laboratorio y con histopatología correspondiente a las lesiones urticarianas del PA e IFD que confirmó el diagnóstico presuntivo clínico. El mismo tuvo muy buena respuesta al tratamiento con corticoides sistémicos, con remisión completa rápida y sin secuelas cicatrizales ni recaídas hasta el momento

We present a 4-month-old with a typical clinical picture of PA, with the characteristic blisters and usually located in less than one year (predominantly on palms and soles and respect for the diaper area) with leukocytosis and eosinophilia laboratory tests and histopathology for the PA urticarial lesions and IFD confirmed clinical presumptive diagnosis. He himself had very good response to treatment with systemic corticosteroids, with complete remission quickly and without sequelae or relapse scarring so far

Bullous pemphigoid: course of the disease. A study of 25 patients

Different studies on the course of bullous pemphigoid have suggested that there may be significant variations in the survival of these patients based on their ethnicity or region of origin. Because of the lack of studies on patients with bullous pemphigoid in the Caribbean, specifically in Puerto Rico, a retrospective analysis about the outcome of patients with this disease in the region was performed. Twenty-five (25) patients were included in this study. The patients were diagnosed as bullous pemphigoid by clinical, histopathologic and immunopathologic assessment. Medical records were reviewed and those patients that were alive were contacted and examined on a follow up visit. Nine were men and sixteen were women; the age at the time of the diagnosis ranged from 54 to 90 year-old (mean 72); 13 were alive after at least 5 years of follow-up and 12 had died. The age at the moment of death ranged from 67 to 95 year-old (mean 83). Of those patients living, 85 were in remission, while 15 had active disease. In general, patients had a disease of mild severity, but, still, 68 of them received systemic corticosteroid therapy. Five of patients had to be admitted due to disease; two of them died while at the hospital. This data suggests that bullous pemphigoid in Puerto Ricans is a disease with a relatively benign course, but which can be fatal in the elderly, especially, if it requires admission to the hospital and the patient receives high doses of systemic corticosteroids

Penfigoide ampollar de la infancia / Bullous pemphogoid in childhood

El penfigoide ampollar es una enfermedad adquirida que usualmente afecta a adultos.Es rara en niños y clínicamente puede ser indistinguible de otras enfermedades ampollares.Presentamos un caso de penfigoide ampollar en una niña de 3 meses de vida con ampollas generalizadas