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1.
Expert Rev Clin Immunol ; 19(11): 1407-1417, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37707350

RESUMO

INTRODUCTION: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disorder in older adults. There is increasing evidence that BP has connections with renal diseases, such as glomerulopathy and neoplasm; it is also linked to the receipt of renal replacement therapy. AREAS COVERED: In this review, we summarize the current evidence that BP is a comorbidity of common renal diseases. Furthermore, our exploration of the characteristics and possible mechanisms underlying these connections provides insights that may facilitate the prevention, diagnosis, and management of BP. EXPERT OPINION: There is mounting proof that BP is not just a skin immunological disorder but rather a systemic immune-mediated illness. Quantities of case reports focused on BP as a renal disease comorbidity and the coexistence of them is not accidental. However, the underlying mechanisms are still needed to be investigated. Clinicians should be alert to the comorbidities in order to facilitate effective treatment and improve patient prognosis.


Assuntos
Penfigoide Bolhoso , Humanos , Idoso , Penfigoide Bolhoso/epidemiologia , Penfigoide Bolhoso/terapia , Penfigoide Bolhoso/diagnóstico , Pele , Comorbidade
2.
Complement Med Res ; 30(3): 221-229, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36646063

RESUMO

INTRODUCTION: Autoimmune bullous diseases (AIBD) are a heterogeneous group of rare autoantibody-mediated blistering dermatoses of the skin and/or mucous membranes. Their incidence is around 20 new cases per million inhabitants per year in Germany. Patients with chronic, oncological, or rare diseases often urge for a holistic therapeutic approach that includes complementary and alternative medicine (CAM). So far, only few contradictory reports on CAM in pemphigoid or pemphigus disease exist. The purpose of this study was to determine the frequency, motives, and satisfaction with the use of alternative treatments in patients with AIBD and to provide a basis for further investigation. METHODS: We used a structured online questionnaire, consisting of 20 questions to survey patients with AIBD and their relatives. The German pemphigus and pemphigoid self-help groups were responsible for distributing anonymized questionnaires. In total, we recovered 97 questionnaires, 63 of which met full inclusion criteria (24 males and 39 females). RESULTS: Of the included participants, more than half had a currently active disease. Of all patients, 58.7% stated that they had used CAM at least once. Women were more likely to use CAM, whereas age and education showed no association to CAM use. The main motives for using CAM were "doing something for oneself" and "opportunity to contribute to treatment" (38.1% each). The internet (23.8%) was the most common source of information, and vitamins were the most frequently used therapy (49.2%). CONCLUSION: Our results provide new insights into the demand for CAM within this rare disease patient group. Physicians should be aware of these methods to meet patient needs but also be able to identify potential barriers such as risks and interactions.


Assuntos
Penfigoide Bolhoso , Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Terapias Complementares , Doenças Raras , Estudos de Coortes , Penfigoide Bolhoso/epidemiologia , Penfigoide Bolhoso/terapia , Estilo de Vida
3.
Prensa méd. argent ; 107(5): 258-263, 20210000. tab, ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1359193

RESUMO

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


Assuntos
Humanos , Masculino , Idoso de 80 Anos ou mais , Dermatopatias/imunologia , Prednisona/uso terapêutico , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/terapia , Inibidores da Dipeptidil Peptidase IV/uso terapêutico
4.
Rev. enferm. UERJ ; 28: e35054, jan.-dez. 2020.
Artigo em Inglês, Português | BDENF, LILACS | ID: biblio-1117622

RESUMO

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.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Vaselina/uso terapêutico , Bandagens , Dermatopatias Vesiculobolhosas/terapia , Penfigoide Bolhoso/terapia , Pênfigo/terapia , Limitação da Mobilidade , Brasil , Lógica Fuzzy , Úlcera por Pressão/prevenção & controle , Prevenção Secundária , Ensaios Clínicos Controlados não Aleatórios como Assunto , Hospitais Públicos , Pacientes Internados , Cuidados de Enfermagem
5.
Front Immunol ; 11: 591971, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33505392

RESUMO

Pemphigus and pemphigoid diseases are autoimmune bullous diseases characterized and caused by autoantibodies targeting adhesion molecules in the skin and/or mucous membranes. Personalized medicine is a new medical model that separates patients into different groups and aims to tailor medical decisions, practices, and interventions based on the individual patient`s predicted response or risk factors. An important milestone in personalized medicine in pemphigus and pemphigoid was achieved by verifying the autoimmune pathogenesis underlying these diseases, as well as by identifying and cloning several pemphigus/pemphigoid autoantigens. The latter has become the basis of the current, molecular-based diagnosis that allows the differentiation of about a dozen pemphigus and pemphigoid entities. The importance of autoantigen-identification in pemphigus/pemphigoid is further highlighted by the emergence of autoantigen-specific B cell depleting strategies. To achieve this goal, the chimeric antigen receptor (CAR) T cell technology, which is used for the treatment of certain hematological malignancies, was adopted, by generating chimeric autoantigen receptor (CAAR) T cells. In addition to these more basic science-driven milestones in personalized medicine in pemphigus and pemphigoid, careful clinical observation and epidemiology are again contributing to personalized medicine. The identification of clearly distinct clinical phenotypes in pemphigoid like the non-inflammatory and gliptin-associated bullous pemphigoid embodies a prominent instance of the latter. We here review these exciting developments in basic, translational, clinical, and epidemiological research in pemphigus and pemphigoid. Overall, we hereby aim to attract more researchers and clinicians to this highly interesting and dynamic field of research.


Assuntos
Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/terapia , Pênfigo/diagnóstico , Pênfigo/terapia , Medicina de Precisão , Animais , Autoantígenos/imunologia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/etiologia , Doenças Autoimunes/terapia , Autoimunidade , Biomarcadores , Diagnóstico Diferencial , Gerenciamento Clínico , Suscetibilidade a Doenças , Humanos , Técnicas de Diagnóstico Molecular , Penfigoide Bolhoso/etiologia , Pênfigo/etiologia , Pênfigo/metabolismo , Medicina de Precisão/métodos , Medicina de Precisão/tendências
6.
Am J Obstet Gynecol ; 222(2): 114-122, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31513780

RESUMO

Vaginitis is one of the most common causes of patient visits to gynecologists, primary care providers, and urgent care centers. However, many women leave without a clear diagnosis or experience recurrent symptoms despite treatment. The 3 most common etiologies of vaginitis are trichomonas, bacterial vaginosis, and vulvovaginal candidiasis, which account for an estimated 70% of cases. The remaining 30% may be related to other causes of vaginitis, including atrophic vaginitis, desquamative inflammatory vaginitis, and vaginal erosive disease. The purpose of this review is to describe the noncandidal causes of acute and recurrent vaginitis, with the goal of improving the likelihood of accurate diagnosis as well as efficient and effective therapy. We excluded candidal vaginitis from our review because there was a recently published review on this topic in the Journal. The clinical presentation and evaluation of patients with symptoms of vaginitis can be triaged into 1 of 2 diagnostic pathways: noninflammatory and inflammatory vaginitis. The most common noninflammatory cause is bacterial vaginosis. Features such as irritation, purulent discharge, and the presence of polymorphonuclear neutrophils are more suggestive of an inflammatory process. Trichomoniasis is the most common cause of inflammatory vaginitis. Other well-described forms of inflammatory vaginitis include atrophic vaginitis, desquamative inflammatory vaginitis, and erosive disease. We present a review of the pathogenesis, symptoms, examination findings, diagnostic testing, and treatment for each of these causes of noncandidal vaginitis.


Assuntos
Anti-Infecciosos/uso terapêutico , Vaginite Atrófica/diagnóstico , Candidíase Vulvovaginal/diagnóstico , Vaginite por Trichomonas/diagnóstico , Vaginose Bacteriana/diagnóstico , Administração Intravaginal , Administração Oral , Anti-Inflamatórios/uso terapêutico , Vaginite Atrófica/terapia , Clindamicina/uso terapêutico , Desidroepiandrosterona/uso terapêutico , Diagnóstico Diferencial , Terapia de Reposição de Estrogênios , Estrogênios/uso terapêutico , Feminino , Humanos , Hidrocortisona/uso terapêutico , Inflamação , Líquen Plano/diagnóstico , Líquen Plano/terapia , Metronidazol/análogos & derivados , Metronidazol/uso terapêutico , Penfigoide Mucomembranoso Benigno/diagnóstico , Penfigoide Mucomembranoso Benigno/terapia , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/terapia , Pênfigo/diagnóstico , Pênfigo/terapia , Tamoxifeno/análogos & derivados , Tamoxifeno/uso terapêutico , Tinidazol/uso terapêutico , Vaginite por Trichomonas/terapia , Vaginite/diagnóstico , Vaginite/terapia , Vaginose Bacteriana/terapia
7.
Dermatol Clin ; 37(2): 215-228, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30850044

RESUMO

The treatment of refractory autoimmune blistering diseases (AIBDs) has always been a challenge. Because randomized controlled trials are lacking, treatment has been based on analysis of anecdotal data. The last 2 decades has seen the use of rituximab become a conventional treatment in the therapeutic armamentarium of AIBDs, leading to its Food and Drug Administration indication for pemphigus vulgaris in 2018. We review the current updated data on the use of rituximab including dosing, protocols, and its role in the algorithm of AIBDs. In addition, we discuss several promising novel emerging therapeutic agents for AIBDs.


Assuntos
Doenças Autoimunes/terapia , Imunossupressores/uso terapêutico , Imunoterapia Adotiva , Plasmaferese , Inibidores de Proteínas Quinases/uso terapêutico , Dermatopatias Vesiculobolhosas/terapia , Tirosina Quinase da Agamaglobulinemia/antagonistas & inibidores , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Doenças Autoimunes/imunologia , Dermatite Herpetiforme/imunologia , Dermatite Herpetiforme/terapia , Epidermólise Bolhosa Adquirida/imunologia , Epidermólise Bolhosa Adquirida/terapia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Penfigoide Gestacional/imunologia , Penfigoide Gestacional/terapia , Penfigoide Mucomembranoso Benigno/imunologia , Penfigoide Mucomembranoso Benigno/terapia , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/terapia , Pênfigo/imunologia , Pênfigo/terapia , Gravidez , Rituximab/uso terapêutico , Dermatopatias Vesiculobolhosas/imunologia , Proteínas Quinases p38 Ativadas por Mitógeno/antagonistas & inibidores
8.
Autoimmun Rev ; 18(4): 349-358, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30738958

RESUMO

Pemphigus diseases (PDs) and mucous membrane pemphigoid (MMP) are a group of immune-mediated mucocutaneous disorders clinically characterized by the formation of blisters, erosions and ulcers. The skin and mucous membranes are predominantly affected, with the oropharyngeal mucosa as the initially involved site. Ocular involvement is also a frequent feature of these diseases. Because of the considerable overlap in their clinical presentations, the diagnosis of PDs vs. MMP can be challenging. A recognition of their specific immunological and histopathologic features is crucial in the differential diagnosis. Treatment modalities include systemically administered corticosteroids, steroid-sparing immunosuppressive agents, and biologic therapies (rituximab, intravenous immunoglobulins, and anti-tumor necrosis factor agents). Topical, oral, conjunctival, or intralesional corticosteroids as well as anti-inflammatory drugs and antibiotics are prescribed as needed.


Assuntos
Penfigoide Mucomembranoso Benigno/diagnóstico , Penfigoide Mucomembranoso Benigno/terapia , Pênfigo/diagnóstico , Pênfigo/terapia , Corticosteroides/uso terapêutico , Diagnóstico Diferencial , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/terapia , Pênfigo/imunologia , Rituximab/uso terapêutico
11.
Rev. chil. dermatol ; 34(1): 32-35, 2018. ilus
Artigo em Espanhol | LILACS | ID: biblio-965818

RESUMO

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.


Assuntos
Humanos , Feminino , Idoso , Terapia Ultravioleta/métodos , Penfigoide Bolhoso/terapia , Biópsia , Ensaio de Imunoadsorção Enzimática , Metotrexato/uso terapêutico , Penfigoide Bolhoso/patologia , Terapia Combinada
12.
Acta Dermatovenerol Croat ; 25(3): 255-256, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29252183

RESUMO

Dear Editor, Bullous pemphigoid (BP), a relatively common autoimmune blistering disease in the elderly, is characterized by large, tense bullae on urticarial, erythematous, or normal skin. However, atypical BP with polymorphic clinical presentations is rarely encountered, leading to misdiagnosis and delayed treatments (1). BP with lesions resembling erythema gyratum repens or figurate erythema has been regarded as a paraneoplastic phenomenon (1). Herein we report a case with erythema annulare centrifugum-like presentation of BP without evidence of underlying malignancy. A 64-year-old woman first presented with multiple large, tense bullae on the trunk and four extremities. She was diagnosed with BP according to the typical clinical, histopathological, and direct immunofluorescence findings. There were no annular lesions at that time. After a treatment course of systemic corticosteroids and azathioprine, the cutaneous symptoms were controlled. One year after discontinuing her medications, a pruritic bullous eruption reappeared with several annular erythematous plaques (Figure 1, a). The patient reported no mucosal involvement and took no new medications before the onset of skin lesions. On physical examination, multiple circular and arcuate erythematous lesions with slightly raised borders were seen on the trunk and both legs. Some erosions and tiny vesicles were noted on the erythematous edges. There were no other systemic symptoms or abnormalities. Laboratory studies, including complete blood count, liver and renal function tests, electrolytes, antinuclear antibody, complement levels, anti-Ro and anti-La antibodies, urine routine, stool routine, and chest X-ray, were normal. The biopsy specimen obtained from the rim of the annular lesions revealed slight vacuolar change at the dermoepidermal junction and perivascular and interstitial lymphocytic infiltration with numerous eosinophils in the upper dermis (Figure 1, b). Direct immunofluorescence showed linear deposits of immunoglobulin G (IgG) and C3 along the basement membrane (Figure 1, c). Histopathological features and immunofluorescence examinations were consistent with BP. There was no evidence of hematological or solid malignancy from further imaging and laboratory testing. The patient was started on oral prednisolone 30 mg/day and azathioprine 150 mg/day, with significant improvement over the following month. Complete regression of all skin lesions was achieved two months later, so the prednisolone dose was gradually tapered and then ceased. Under maintenance monotherapy of azathioprine 100 mg/day, there were no signs of BP recurrence or malignant disease during the one-year follow-up period. The annular erythema variant of BP is extremely rare. Therefore, in this case, erythema multiforme, subacute cutaneous lupus erythematosus, erythema annulare centrifugum, and urticarial vasculitis should be considered in the clinical differential diagnoses. Pathological features and immunofluorescence results can clearly rule out these possibilities. Until now, only 13 cases of BP presenting as annular erythema had been documented in the English literature, described as figurate erythema-like, erythema gyratum repens-like, or erythema annulare centrifugum-like manifestations (1-3). An association with internal malignancy in patients with these types of lesions had been reported (1). Nevertheless, as in most previous case reports (3), malignant diseases were not found in our patient. The precise mechanism of the annular erythema form of BP is unknown. Some authors considered it a variant of pre-bullous phase lesions, usually presenting as itchy erythematous patches or urticarial plaques (4). Based on this case, however, this assumption is less likely because the annular, erythema annulare centrifugum-like skin lesions appeared one year after the initial onset of bullous eruption, and simultaneously with the exacerbation of the bullous phase of BP. The exact pathogenesis of annular BP may be similar to that in erythema annulare centrifugum. Further investigations are warranted to clarify this issue. It should be noted that an erythema annulare centrifugum-like or figurate erythema-like manifestation in the absence of underlying malignancy can occasionally be a feature of BP. Making the correct diagnosis may be difficult if there is no concurrent bullous presentation. Clinicians should be vigilant for the development of this type of BP. The histological and direct immunofluorescence findings and the detection of circulating autoantibodies by indirect immunofluorescence or enzyme-linked immunosorbent assay remain crucial tools for establishing a definitive diagnosis.


Assuntos
Eritema/diagnóstico , Penfigoide Bolhoso/diagnóstico , Dermatopatias Genéticas/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-Idade , Penfigoide Bolhoso/terapia
13.
Rev Prat ; 67(10): 1080-1083, 2017 Dec.
Artigo em Francês | MEDLINE | ID: mdl-30512605

RESUMO

Bullous pemphigoid, a dermatosis of the elderly. Bullous pemphigoid is the most common autoimmune bullous disease and affects almost exclusively the elderly. Its occurrence is related to the presence of pathogenic autoantibodies directed against structural proteins (BP180 and BP230) of the protein adhesion complex of the dermo-epidermal junction: the hemi-desmosome. Bullous pemphigoid is classically characterized by pruritus and the appearance of blisters on an inflammatory background with a symmetrical topography: thighs, arms and trunk. Blisters eventually break, leading to erosions. Mucosal involvement is rare. Histology of a cutaneous biopsy finds a subepidermal blister containing eosinophils. Direct immunofluorescence confirms the diagnosis by the presence of linear deposits of IgG and C3 along the epidermal basement membrane. The reference treatment is the superpotent topical corticosteroid therapy (clobetasol propionate).


La pemphigoïde bulleuse, une dermatose du sujet âgé. La pemphigoïde bulleuse est la dermatose bulleuse auto-immune la plus fréquente et elle touche quasi exclusivement la personne âgée. Sa survenue est liée à la présence d'autoanticorps pathogènes dirigés contre des protéines de structure (BP180 et BP230) du complexe protéique d'adhésion de la jonction dermo-épidermique : l'hémidesmosome. La pemphigoïde bulleuse se manifeste classiquement par un prurit et l'apparition de bulles sur fond inflammatoire avec une topographie symétrique : cuisses, bras, tronc. Les bulles finissent par se rompre, laissant la place à des érosions. L'atteinte muqueuse est rare. L'histologie d'une biopsie cutanée montre une bulle sous-épidermique contenant des polynucléaires éosinophiles. L'immuno- fluorescence directe affirme le diagnostic par la présence de dépôts linéaires d'immunoglobuline de type G et de C3 le long de la membrane basale épidermique. Le traitement de référence est la corticothérapie locale très forte (propionate de clobétasol).


Assuntos
Doenças Autoimunes , Penfigoide Bolhoso , Dermatopatias Vesiculobolhosas , Idoso , Autoanticorpos , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/terapia , Humanos , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/terapia , Pele
14.
J Cutan Med Surg ; 20(6): 570-572, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27207351

RESUMO

INTRODUCTION: Itching nodules and papules are common findings. A rare but important differential diagnosis is the nodular subtype of bullous pemphigoid. METHODS AND RESULTS: The investigators report a female patient presenting with strongly itching papules disseminated over her extremities and trunk. Physical examination revealed multiple erythematous, mostly excoriated papules and nodules on her back, abdomen, and extremities. Histology showed changes compatible with prurigo lesion, and immunofluorescence showed positive results for BP180 and BP230. Considering these clinical, histologic, and immunofluorescence findings, the diagnosis of a nodular subtype of bullous pemphigoid was made. The patient showed healing of lesions under a combination therapy with systemic psoralen and ultraviolet A, topical application of corticosteroids, and systemic therapy with azathioprine and prednisolone. DISCUSSION: Pemphigoid nodularis represents the rare prurigo variant of bullous pemphigoid. Typically, lesions show the same immunopathologic and histologic features as in common bullous pemphigoid but mostly without the characteristic clinical finding of bullae.


Assuntos
Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/patologia , Idoso de 80 Anos ou mais , Autoantígenos/análise , Distonina/análise , Feminino , Humanos , Colágenos não Fibrilares/análise , Penfigoide Bolhoso/terapia , Prurido/etiologia , Colágeno Tipo XVII
16.
Med Hypotheses ; 85(4): 412-4, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26152650

RESUMO

Mucous membrane pemphigoid (MMP) is a subtype of autoimmune subepidermal blistering diseases characterized by autoantibodies to structural components of the hemidesmosome primarily affecting mucous membranes. Inflammation-related progressive scarring can lead to serious complications, including blindness, and the disease may be associated with malignancy. Conventional immunosuppressive treatment is often insufficiently effective and limited due to side effects, warranting new therapeutic options ideally targeting both inflammation and extensively recalcitrant cicatrization. Heat shock protein 90 (Hsp90) is a cell stress-inducible chaperone required for the function of a large number of client proteins, and its pharmacological inhibition has proven to be effective and relatively safe in patients with cancer. Recent observations also suggest a promising role of Hsp90 as drug target in preclinical in vivo murine models of autoimmune diseases such as subepidermal bullous and fibrotic autoimmune disorders comprising epidermolysis bullosa acquisita and systemic sclerosis, respectively, which exhibit some pathophysiological features reminiscent of MMP. This article thus hypothesizes that Hsp90 blockade could represent a double-edged sword in MMP treatment by targeting pathogenic factors of inflammatory blister and fibrosis formation. Moreover, Hsp90 inhibitors could even be proclaimed as a triple-edged sword in case of an underlying malignancy. Future studies investigating the role of Hsp90 in MMP are needed to clarify whether Hsp90 inhibition could become a novel treatment approach for patients with this potentially devastating disease.


Assuntos
Proteínas de Choque Térmico HSP90/antagonistas & inibidores , Mucosa/fisiopatologia , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/terapia , Animais , Autoanticorpos/imunologia , Doenças Autoimunes/imunologia , Autoimunidade/imunologia , Epiderme/imunologia , Fibrose/fisiopatologia , Proteínas de Choque Térmico HSP90/química , Humanos , Imunoglobulina G/química , Imunossupressores/química , Inflamação , Camundongos , Modelos Teóricos , Chaperonas Moleculares , Neoplasias/imunologia , Neoplasias/fisiopatologia , Pele/fisiopatologia
17.
Autoimmun Rev ; 13(4-5): 482-9, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24434358

RESUMO

Blistering skin diseases are a group of autoimmune disorders that are characterized by autoantibodies against structural proteins of the epidermis or the dermal-epidermal junction and clinically by blisters and erosions on skin and/or mucous membranes. Since clinical criteria and histopathological characteristics are not sufficient for diagnosis, direct immunofluorescence microscopy of a biopsy specimen or serological tests are needed for exact diagnosis. The differentiation between the various disorders became more important since prognosis as well as different treatment options are nowadays available for the various diseases. Moreover, some bullous diseases may indicate the presence of an underlying malignancy. The detection of serum autoantibodies have been shown to correlate with disease activity and thus may be helpful in deciding treatment options for these patients.


Assuntos
Doenças Autoimunes/diagnóstico , Penfigoide Bolhoso/diagnóstico , Autoanticorpos/sangue , Doenças Autoimunes/imunologia , Doenças Autoimunes/patologia , Doenças Autoimunes/terapia , Epiderme/imunologia , Epiderme/patologia , Humanos , Imunoglobulina A/imunologia , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/patologia , Penfigoide Bolhoso/terapia , Prognóstico
19.
Lancet ; 381(9863): 320-32, 2013 Jan 26.
Artigo em Inglês | MEDLINE | ID: mdl-23237497

RESUMO

Pemphigoid diseases are a group of well defined autoimmune disorders that are characterised by autoantibodies against structural proteins of the dermal-epidermal junction and, clinically, by tense blisters and erosions on skin or mucous membranes close to the skin surface. The most common of these diseases is bullous pemphigoid, which mainly affects older people and the reported incidence of which in Europe has more than doubled in the past decade. Prognosis and treatments vary substantially between the different disorders and, since clinical criteria are usually not sufficient, direct immunofluorescence microscopy of a perilesional biopsy specimen or serological tests are needed for exact diagnosis. In eight pemphigoid diseases the target antigens have been identified molecularly, which has allowed the development of standard diagnostic assays for detection of serum autoantibodies-some of which are commercially available. In this Seminar we discuss the clinical range, diagnostic criteria, diagnostic assay systems, and treatment options for this group of diseases.


Assuntos
Penfigoide Bolhoso , Animais , Autoanticorpos/análise , Humanos , Penfigoide Mucomembranoso Benigno/imunologia , Penfigoide Mucomembranoso Benigno/patologia , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/fisiopatologia , Penfigoide Bolhoso/terapia
20.
South Med J ; 105(11): 600-6, 2012 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-23128804

RESUMO

Diseases of elderly adults are becoming increasingly important as life expectancy gradually rises worldwide. To promote healthy aging, it is important to understand the skin changes associated with aging. This review focuses on the special considerations for some of the more common dermatological disorders in elderly adults and examines presentation, contributing factors, and association with systemic diseases.


Assuntos
Dermatopatias , Idoso , Idoso de 80 Anos ou mais , Dermatite de Contato/diagnóstico , Dermatite de Contato/terapia , Eczema/diagnóstico , Eczema/etiologia , Eczema/terapia , Herpes Zoster/diagnóstico , Herpes Zoster/terapia , Humanos , Ceratose Seborreica/diagnóstico , Ceratose Seborreica/terapia , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/terapia , Lesões Pré-Cancerosas/diagnóstico , Lesões Pré-Cancerosas/terapia , Prurido/etiologia , Prurido/terapia , Envelhecimento da Pele , Dermatopatias/diagnóstico , Dermatopatias/etiologia , Dermatopatias/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Insuficiência Venosa/diagnóstico , Insuficiência Venosa/terapia
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