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

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)

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.

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: 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.

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

Dyshidrosiform pemphigoid localized on the hands in a child: a rare occurrence

Abstract: Dyshidrosiform pemphigoid is an acquired autoimmune variant of bullous pemphigoid with persistent vesicobullous eruptions localized on the palms or soles, or both. It generally occurs in the elderly and is rarely reported in childhood. Hereby, we describe the first case of dyshidrosiform pemphigoid in a 12-year-old child, which was limited to the dorsal hands and treated successfully with dapsone (diaminodiphenyl sulfone). Along with this report, we also review the clinical features of various types of dyshidrosiform pemphigoid.

Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient

Abstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.

Combined acute interstitial pneumonitis and pancytopenia induced by low-dose methotrexate in a hemodialysis patient treated for bullous pemphigoid

Methotrexate has been widely used for many years in the treatment of a variety of diseases. Acute pneumonitis and bone marrow suppression are very serious side effects in methotrexate treatment. A 48-year-old man with end-stage renal disease undergoing chronic hemodialysis developed combined acute pneumonitis and pancytopenia after a cumulative dose of 20 mg methotrexate for bullous pemphigoid. Continuous renal replacement therapy (CRRT) can effi ciently decrease serum methotrexate concentration. A rapid improvement of clinical symptoms and resolution of pulmonary opacifi cation were found after CRRT. Blood cell counts returned to normal after component blood transfusion and cytokine supportive therapy. Patients with impaired renal function are at high risk of methotrexate toxicity, and low-dose methotrexate should be prescribed with great caution.

Bullous pemphigoid and comorbidities: a case-control study in Portuguese patients

BACKGROUND: aAlthough rare, bullous pemphigoid (BP) is the most common autoimmune blistering disease. Recent studies have shown that patients with bullous pemphigoid are more likely to have neurological and psychiatric diseases, particularly prior to the diagnosis of bullous pemphigoid. OBJECTIVE: The aims were: (i) to evaluate the demographic and clinical features of bullous pemphigoid from a database of patients at a Portuguese university hospital and (ii) to compare the prevalence of comorbid conditions before the diagnosis of bullous pemphigoid with a control group. METHODS: Seventy-seven patients with bullous pemphigoid were enrolled in the study. They were compared with 176 age- and gender-matched controls, which also had the same inpatient to outpatient ratio, but no history of bullous or cutaneous malignant disease. Univariate and multivariate analyses were used to calculate odds ratios for specific comorbid diseases. RESULTS: At least one neurologic diagnosis was present in 55.8% of BP patients compared with 20.5% controls (p<0.001). Comparing cases to controls, stroke was seen in 35.1 vs. 6.8%, OR 8.10 (3.80-17.25); dementia in 37.7 vs. 11.9%, OR 5.25 (2.71-10.16); and Parkinson's disease in 5.2 vs. 1.1%, OR 4.91 (0.88-27.44). Using multivariate analysis, all diseases except Parkinson's retained their association with BP. Patients under systemic treatment were eight times more likely to have complications than those treated with topical steroids (p< 0.017). CONCLUSIONS: The results of this study substantiate the association between BP and neurological diseases. In addition, they highlight the potential complications associated with the treatment of BP. .

Bullous pemphigoid in a 3-month-old infant: case report and literature review of this dermatosis in childhood / Penfigoide bolhoso em lactente de 3 meses: relato de caso e revisao de literatura da dermatose na infancia

Bullous pemphigoid is an autoimmune subepidermal blistering dermatosis that is uncommon in childhood. We report a case of a female infant, 3 months old, which presented clinical and laboratory data for the confirmatory diagnosis of bullous pemphigoid. The authors used immunohistochemical staining for collagen type IV that allowed the differentiation of bullous pemphigoid from other subepidermal bullous diseases. Opportunely we review the clinical, immunological, therapeutic and prognostic features of this pathology in children.

O penfigoide bolhoso é uma dermatose bolhosa autoimune subepidérmica, incomum na infância. Relatamos um caso de lactente feminina, com 3 meses de idade, que apresentou dados clínicos e laboratoriais confirmatórios para o diagnóstico de penfigoide bolhoso. Os autores utilizaram a coloração de imuno-histoquímica para o colágeno tipo IV que permitiu a diferenciação do penfigoide bolhoso de outras buloses subepidérmicas. Oportunamente, revisamos as características clínicas, imunológicas, terapêuticas e prognósticas da patologia na criança.

Penfigoide bolhoso no adulto mais jovem: relato de três casos / Bullous pemphigoid in younger adults: three case reports

O penfigoide bolhoso é uma dermatose bolhosa autoimune subepidérmica, mais comumente observada na população idosa (acima dos 70 anos). Autoanticorpos são formados contra antígenos específicos da zona de membrana basal: BP180 e BP230 (proteínas do hemidesmossomo). Apresentamos três casos de penfigoide bolhoso, em adultos com menos de 50 anos de idade, destacan do as peculiaridades clínicas na faixa etária mais jovem.

Bullous pemphigoid is an autoimmune subepidermal bullous dermatosis more commonly observed in the elderly (over 70 years old). Autoantibodies are produced for specific antigens of the epidermal basement membrane zone: BP 180 and BP 230 (hemidesmosome proteins). We report three cases of bullous pemphigoid in adults younger than 50 years old, discussing the clinical characteristics of the disease in younger patients.

Penfigoide ampollar: análisis retrsopectivo de 45 casos / Bullous pemphigoid: a retrospective analysis of 45 cases

Introducción. El penfigoide ampollar (PA) es una enfermedad autoinmune que afecta con mayor frecuencia a personas de edad avanzada.Objetivos. Identificar la edad de presentación, sexo y variantes clínicas del PA. Establecer la asociación con otras enfermedades, la respuesta terapéutica y comparar los resultados con las estadísticas publicadas.Materiales y métodos. Se realizó un análisis retrospectivo y descriptivo de los pacientes que consultaron en el Servicio de Dermatología del Policlínico Bancario en el período comprendido entre agosto de 1995 y el mismo mes de 2010. Se incluyeron aquellos pacientes con diagnóstico de PA. La recolección de datos se obtuvo de los registros histológicos y de las correspondientes historias clínicas.Resultados. Se registraron 45 pacientes; 25 (55,5%) femeninos y 20 (44,5%) masculinos. La edad media fue de 75,9 años. Variantes clínicas: 35 pacientes (77,7%) clásica; 5 (11,1%) localizada; 1(2,2%) vesicular; 2 (4,4%) nodular. Asociación con DBT: 15 pacientes (33,3%); HTA: 29 (64,4%); otras enfermedades sistémicas: 19 (42,2%); enfermedades dermatológicas: 7 (15,5%). La respuesta terapéutica fue buena.Conclusiones. La edad promedio al momento del diagnóstico fue avanzada. No hallamos marcada diferencia en cuanto a la distribución por sexo. La forma clásica predominó sobre las otras variantes clínicas. La asociación con enfermedades sistémicas podría deberse a que estas patologías se dan con mayor frecuencia en el mismo grupo etario. La asociación con enfermedades dermatológicas fue conpsoriasis (la de mayor representación). La respuesta terapéutica fue altamente satisfactoria. En general, nuestros datos son similares a las estadísticas publicadas.

Epidermólise bolhosa adquirida inflamatória: relato de caso / Inflammatory epidermolysis bullosa acquisita: case report

Apresenta-se caso de epidermólise bolhosa adquirida inflamatória. Paciente do sexo masculino, 53 anos, há seis meses com erupção vesicobolhosa pruriginosa sobre base eritematosa no tronco, axilas e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos. A imunofluorescência direta revelou depósitos lineares de IgG, IgA, IgM e C3 na zona da membrana basal, sendo a imunofluorescência indireta e o Salt Split Skin indireto negativos. Anticorpos antinucleares não reagentes. Houve melhora do quadro com prednisona e cicatrização de algumas lesões com formação de milia. Trata-se de apresentação rara de epidermólise bolhosa adquirida, com lesões iniciais predominantemente inflamatórias.

We report a case of an inflammatory variant of epidermolysis bullosa acquisita in a 53-year-old male, with itching blistering eruption on the trunk, armpits and limbs for six months. The skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed linear depositions of IgG, IgA, IgM and C3 at the basement membrane; indirect immunofluorescence and salt Split Skin were negative. Antinuclear antibodies were also negative. Improvement of the blisters followed treatment with systemic corticotherapy and some lesions healed with milia. This is a rare presentation of epidermolysis bullosa acquisita, with inflammatory lesions at first.