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2.
An. bras. dermatol ; 95(5): 670-671, Sept.-Oct. 2020. graf
Article in English | LILACS, ColecionaSUS | ID: biblio-1130944
3.
Rev. Asoc. Méd. Argent ; 133(1): 29-33, mar. 2020.
Article in Spanish | LILACS | ID: biblio-1097707

ABSTRACT

Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)


Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)


Subject(s)
Humans , Adult , Anus Diseases/diagnosis , Anus Diseases/therapy , Patient Care Team , Skin Diseases, Vesiculobullous/diagnosis , Skin Diseases, Vesiculobullous/therapy , Dermatitis Herpetiformis/diagnosis , Dermatitis Herpetiformis/therapy , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/therapy , Pemphigus/diagnosis , Pemphigus/therapy , Stevens-Johnson Syndrome/diagnosis , Stevens-Johnson Syndrome/therapy , Pemphigus, Benign Familial/diagnosis , Pemphigus, Benign Familial/therapy , Dermatitis, Contact/diagnosis , Dermatitis, Contact/therapy
4.
Rev. argent. dermatol ; 101(1): 111-120, mar. 2020. graf
Article in Spanish | LILACS | ID: biblio-1125812

ABSTRACT

Resumen El pénfigo foliáceo es una enfermedadversículo ampollar autoinmune poco frecuente,caracterizada histopatológicamente por acantólisis, inducida por la presencia de autoanticuerpos frente a la desmogleína 1. El desprendimiento se localiza en los estratos más superficiales de la epidermis produciendo ampollas frágiles y erosiones. Se clasifica en endémico y no endémico o esporádico. Dentro de este último podemos encontrar una variedad localizada y una generalizada. Presentamos el caso de un paciente, con diagnósticoy confirmación histopatológica de pénfigo foliáceo y realizamos una breve revisión de la literatura.


Summary Foliaceus pemphigus is an infrequent autoimmune blistering verse disease characterized histopathologically by acantholysis, induced by the presence of autoantibodies against desmoglein 1. The detachment is located in the most superficial layers of the epidermis produces fragile blisters and erosions. It is classified as endemic, and not endemic or sporadic. Within the latter we can find a localized and a generalized variety. Presents the case of a patient, diagnosis and histopathological confirmation of a paper and makes a brief review of the literature.


Subject(s)
Humans , Male , Adult , Pemphigus/diagnosis , Pemphigus/drug therapy , Biopsy/methods , Desmoglein 1/agonists
5.
An. bras. dermatol ; 94(4): 388-398, July-Aug. 2019. tab, graf
Article in English | LILACS | ID: biblio-1038294

ABSTRACT

Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.


Subject(s)
Humans , Paraneoplastic Syndromes/pathology , Paraneoplastic Syndromes/therapy , Pemphigus/immunology , Pemphigus/pathology , Pemphigus/therapy , Paraneoplastic Syndromes/immunology , Skin/pathology , Autoantibodies/immunology , Pemphigus/diagnosis , Erythema/diagnosis , Erythema/pathology , Mouth Diseases/diagnosis , Mouth Diseases/pathology
6.
An. bras. dermatol ; 94(3): 264-278, May-June 2019. tab, graf
Article in English | LILACS | ID: biblio-1011108

ABSTRACT

Abstract: Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Pemphigus/diagnosis , Skin/pathology , Autoantibodies/immunology , Surveys and Questionnaires , Pemphigus/classification , Pemphigus/therapy , Pemphigus/epidemiology , Immunoglobulins, Intravenous/therapeutic use , Desmosomes/immunology , Diagnosis, Differential , Immunosuppressive Agents/classification , Immunosuppressive Agents/therapeutic use , Immunotherapy/methods
7.
Rev. Paul. Pediatr. (Ed. Port., Online) ; 37(1): 130-134, Jan.-Mar. 2019. graf
Article in Portuguese | LILACS | ID: biblio-985134

ABSTRACT

RESUMO Objetivo: Relatar um caso de pênfigo neonatal em paciente que manifestou lesões cutâneas extensas e críticas ao nascimento. Descrição do caso: Recém-nascido do sexo masculino com lesões vesicobolhosas extensas em região anterior do tórax e abdome, desde o nascimento. Admitido na ala pediátrica de um hospital para diagnóstico etiológico e tratamento. Com base na história materna e na avaliação clínica, concluiu tratar-se de pênfigo vulgar neonatal. O paciente apresentou evolução satisfatória, sem a necessidade de intervenção farmacológica. Comentários: Os casos descritos na literatura e as referências avaliadas revelam o pênfigo neonatal como uma doença de ocorrência rara, porém cujo conhecimento e diagnóstico precoce têm grande relevância clínica, considerando-se que geralmente se manifesta com lesões epidérmicas extensas e de aspecto crítico, embora apresente curso clínico transitório e benigno, sem necessidade de tratamento específico e sem relação com doença futura.


ABSTRACT Objective: To report on the case of a patient with neonatal pemphigus that had extensive and critical skin lesions at birth. Case description: A newborn male with extensive vesico-bullous lesions on the anterior side of his chest and abdomen at birth. He was admitted to the pediatric ward of a hospital for an etiological diagnosis and for treatment. Based on maternal history and a clinical evaluation, the patient was diagnosed with neonatal vulgar pemphigus. His progression was satisfactory and, in the end, he did not need pharmacological interventions. Comments: The cases reported in the literature and the references evaluated reveal that neonatal pemphigus is rare, but that knowledge about the disease allows for an early diagnosis to be made. This has great clinical relevance considering that the disease usually manifests itself in the form of extensive epidermal lesions, even though it is transient and benign, it does not require specific treatment, and it does not have any relation with possible future diseases.


Subject(s)
Humans , Male , Female , Infant, Newborn , Adult , Infant, Newborn, Diseases/diagnosis , Remission, Spontaneous , Pemphigus/diagnosis , Pemphigus/physiopathology , Infant, Newborn, Diseases/physiopathology , Medical History Taking , Mothers
8.
Rev. medica electron ; 40(6): 2083-2096, nov.-dic. 2018. graf
Article in Spanish | LILACS, CUMED | ID: biblio-978719

ABSTRACT

RESUMEN El pénfigo es una enfermedad autoinmune potencialmente mortal, que causa ampollas y erosiones en la piel y en la membrana mucosa. Las lesiones epiteliales son el resultado de autoanticuerpos que reaccionan con las glicoproteínas desmosomales y están presentes en la superficie celular del queratinocito. La reacción autoinmune contra estas glicoproteínas causa una pérdida de adhesión celular, resultando en la formación de ampollas intraepiteliales. Del 80 al 90 % de los pacientes con pénfigo vulgar, desarrollan trastornos cutáneos y en el 60 % de los casos alteraciones en la mucosa que es el primer o único signo. El diagnóstico de las lesiones en cavidad bucal es fundamental, ya que pueden prevenir su afectación a la piel. Si se establece el tratamiento en su etapa inicial, la enfermedad es más fácil de controlar y aumenta la posibilidad de una remisión temprana del trastorno y mejor calidad de vida. Este reporte de caso mostró a una paciente de 35 años, la que comenzó a presentar lesiones ulceradas en toda la orofaringe, con sensación de ardor e incapacidad para la ingestión de alimentos. El diagnóstico fue pénfigo vulgar (AU).


ABSTRACT Pemphigus is a potentially deadly autoimmune disease causing blisters and erosions in the skin and the mucous membrane. The epithelial lesions are the result of antibodies reacting to desmosomal glycoproteins, and are present in the keratinocytes cellular surface. The autoimmune reaction to these glycoproteins causes a cellular adhesion loss resulting in the formation of intraepithelial blisters. From 80 to 90 % of the patients with vulgar pemphigus develop skin disorders, and 60 % of the cases show mucosa changes as the first or unique sign. The diagnosis of the lesions in oral cavity is essential because it could prevent the skin damage. If the treatment begins in an initial stage, it is easier to control the disease and the possibility of the disorder's early remission and a better life quality increases. This is the report of the case of a female patient, aged 35 years, who presented ulcerated lesions in the entire oropharyngeal region, with itching sensation and inability for food consumption. The diagnosis was vulgar pemphigus (AU).


Subject(s)
Humans , Female , Adult , Skin Diseases/etiology , Surgery, Oral , Pemphigus/etiology , Oral Ulcer/diagnosis , Lymphadenopathy/diagnosis , Skin Diseases/diagnosis , Skin Diseases/drug therapy , Triamcinolone/therapeutic use , Pemphigus/diagnosis , Pemphigus/drug therapy , Adrenal Cortex Hormones/therapeutic use , Lymphadenopathy/pathology , Gingivitis/diagnosis
9.
An. bras. dermatol ; 93(5): 638-650, Sept.-Oct. 2018. tab, graf
Article in English | LILACS | ID: biblio-949951

ABSTRACT

Abstract: Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate. Brazil has the world's largest number of patients, mainly in the Central-West region, but the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, with occurrence of familial cases. Anti-desmoglein-1 autoantibodies are directed against desmosomal structures, with loss of adhesion of the upper layers of the epidermis, causing superficial blisters. The etiology is multifactorial and includes genetic, immune, and environmental factors, highlighting hematophagous insect bites; drug-related factors are occasionally involved. Flaccid blisters readily rupture to yield erosive-crusty lesions that sometimes resemble seborrheic dermatitis, actinic keratosis, and chronic cutaneous lupus erythematosus. The clinical presentation varies from localized to disseminated lesions. Clinical suspicion should be confirmed with histopathological and immunofluorescence tests, among others. The progression is usually chronic, and therapy varies according to clinical presentation, but generally requires systemic corticosteroid therapy associated with adjuvant immunosuppressive treatment to decrease the adverse effects of corticosteroids. Once the disease is under control, many patients remain stable on low-dose medication, and a significant proportion achieve remission.


Subject(s)
Humans , Pemphigus/etiology , Pemphigus/epidemiology , Endemic Diseases , Autoantibodies/immunology , Brazil/epidemiology , Photography , Pemphigus/diagnosis , Pemphigus/pathology , Desmogleins/immunology
10.
Rev. chil. pediatr ; 89(5): 650-654, oct. 2018. graf
Article in Spanish | LILACS | ID: biblio-978138

ABSTRACT

Resumen: Introducción: El pénfigo vulgar es una enfermedad grave y poco frecuente en niños. Su diagnóstico y tratamiento oportuno permite modificar el pronóstico. El objetivo es describir las características clínicas y el abordaje diagnóstico y terapéutico de esta poco frecuente enfermedad vesículo-ampollar autoinmune en niños. Caso clínico: Niño de 2 años, previamente sano. Comenzó con dermato sis generalizada con máximo lesional en región umbilical, miembros y genitales, caracterizada por ampollas, algunas denudadas con sangrado fácil, sin compromiso mucoso ni fiebre. Se diagnosticó impétigo bulloso y se indicó antibioticoterapia tópica y sistémica sin mejoría clínica. Evolucionó con extensión lesional, con compromiso de mucosas oral y anal. El estudio histológico e inmunohistoquí- mico de las lesiones y la piel perilesional confirmó pénfigo vulgar. Se inició tratamiento corticoideo e inmunomodulador con buena respuesta. Conclusiones: Dada la similitud de las manifestaciones del pénfigo vulgar con otras enfermedades infecciosas e inflamatorias de mayor prevalencia, se requiere un alto índice de sospecha para evitar demoras en el diagnóstico y el comienzo del tratamiento. En pacientes con manifestaciones vesículo ampollares de evolución no esperada, es necesaria la intercon sulta por dermatólogo y evaluar la oportunidad de biopsia de la lesión y piel perilesional, para estudio histológico e inmunofluorescencia directa, lo que permitirá la confirmación diagnóstica.


Abstract: Introduction: pemphigus vulgaris is a serious and infrequent disease in children. Its timely diagnosis and treatment allows modifying its prognosis. The objective is to describe its clinical characteristics, and the diagnostic and therapeutic approach of this uncommon autoimmune blistering disease in children. Clinical case: 2-year-old male patient, previously healthy. He initially presented generalized dermatosis with maximum lesion areas at the umbilical region, limbs and genitals; characterized by blisters, some denuded, and of easy bleeding, without mucosal involvement nor fever. Bullous im petigo was diagnosed and topical and systemic antibiotic treatment was started, showing no clinical improvement. He developed extension of the lesions with oral and anal mucosal involvement. The histologic and direct immunofluorescent study of lesions and perilesional skin confirm the diagnosis of pemphigus vulgaris. The patient started treatment with corticosteroids and immunomodulatory agents with good clinical response. Conclusions: Due to the similarity with other more prevalent infectious and inflammatory diseases, a high index of suspicion is required in order to avoid delays in the diagnosis and the start of treatment. In patients with blisters with an unexpected clinical evolu tion, it is necessary to conduct a joint evaluation with a dermatologist and to assess the opportunity of performing a biopsy of the lesion and perilesional skin for histological study and direct immunofluo rescence, which will allow diagnostic confirmation.


Subject(s)
Humans , Male , Child, Preschool , Pemphigus/diagnosis , Pemphigus/drug therapy , Immunosuppressive Agents/therapeutic use
11.
J. oral res. (Impresa) ; 7(9): 432-436, ene. 2, 2018. ilus
Article in English | LILACS | ID: biblio-1121164

ABSTRACT

Pemphigus is a chronic potentially fatal autoimmune disorder that causes blisters and erosions of the skin and oral mucous membrane. most of the cases present oral manifestations as the first clinical sign along with dermal lesions. only 0.5 to 3.2 of cases are reported each year per 1,000,000 population with oral manifestations without dermal participation, and is at times difficult to diagnose. we report a case of oral pemphigus vulgaris in a 20 year old female patient without dermal manifestations treated with oral mini pulse therapy. pénfigo oral tratado con terapia minipulse. resumen: el pénfigo es un trastorno autoinmune crónico potencialmente fatal que causa ampollas y erosiones de la piel y la membrana mucosa oral. la mayoría de los casos presentan manifestaciones orales como el primer signo clínico junto con lesiones dérmicas. solo se reportan de 0.5 a 3.2 casos cada año por cada 1,000,000 de personas con manifestaciones orales sin afectación de la piel, y algunas veces es difícil de diagnosticar. presentamos un caso de pénfigo vulgar oral en un paciente de 20 años, sin manifestaciones cutáneas tratadas con mini terapia del pulso oral.


Subject(s)
Humans , Female , Adult , Young Adult , Skin/pathology , Autoimmune Diseases/drug therapy , Pemphigus/diagnosis , Pemphigus/drug therapy , Mouth Mucosa/injuries , Autoimmune Diseases/therapy , Prednisolone/administration & dosage , Pemphigus/mortality , Pulse Therapy, Drug
12.
Einstein (Säo Paulo) ; 15(2): 220-222, Apr.-June 2017. tab, graf
Article in English | LILACS | ID: biblio-891373

ABSTRACT

ABSTRACT Given the challenge of clinical diagnosis of bullous skin lesions, this report aimed to discuss the histological changes, the presentation and clinical reasoning for diagnosis of these lesions. At the same time, the importance of the pathology was reviewed to identify these clinical scenarios. In this case report, we highlighted the clinical progression of a case of pemphigus foliaceus.


RESUMO Considerando o desafio do diagnóstico clínico de lesões cutâneas de apresentação bolhosa, o presente trabalho procurou discutir as alterações histológicas, a apresentação e o raciocínio clínico para o diagnóstico de tais lesões. Paralelamente, a importância da patologia foi revisada na identificação destes quadros. Neste relato de caso, destaca-se a evolução clínica de um quadro de pênfigo foliáceo.


Subject(s)
Humans , Male , Aged , Acantholysis/pathology , Pemphigus/diagnosis , Keratinocytes/pathology , Skin Diseases, Vesiculobullous/diagnosis , Pemphigus/pathology , Diagnosis, Differential
13.
Acta pediátr. hondu ; 7(2): 663-669, mar. 2017. ilus, tab
Article in Spanish | LILACS | ID: biblio-979714

ABSTRACT

El Lupus Eritematoso sistémico (LES) es una enfermedad autoinmunitaria, multisistémica y episódica, caracterizada por la in amación vascular generalizada y del tejido conectivo, además por la presencia de anticuerpos anti-nucleares (ANA) y anticuerpos anti-DNA de doble cadena. El término pén go; enfermedad ampollar poco frecuente, de etiología autoin-mune, que afecta la piel y mucosas. De ne un grupo de enfermedades autoinmunes de la piel caracterizadas por la presencia de inmuno-globulina G (IgG) dirigida a la super cie de los queratinocitos y ampollas debidas a acantólisis (separación intercelular).Paciente femenina de 16 años de edad, con historia de presentar edema en rodilla derecha, manos y pies acompañada de artralgias de un año de evolución. Además, di cultad para la deambulación de 3 días de evolución. Presentó lesiones ampollosas en mucosa oral y labio superior con uyentes y se extienden a piel circundante. Las articulaciones metacarpofa-lángicas de manos estaban edematizadas, dolorosas, con leve deformidad y limitación funcional. Lasampollas inicialmente localiza-das en cara y extremidades inferiores se fueron extendiendo a miembros inferiores, acompa-ñado de eritema palmar bilateral. Se le diag-nosticó Pén go Vulgar y Lupus Eritematoso Sistémico. Este último, por presentar criterios laboratoriales compatibles con LES: leucope-nia, ANA positivo, nefropatía y artritis. Se inició tratamiento con prednisona 50 mg vía oral cada día (1 mg/kg/día)...(AU)


Subject(s)
Humans , Female , Adolescent , Autoimmune Diseases , Pemphigus/diagnosis , Joint Diseases , Lupus Erythematosus, Systemic/complications
14.
Rev. ADM ; 73(1): 28-32, ene.-feb.2016. ilus
Article in Spanish | LILACS | ID: lil-781839

ABSTRACT

El pénfigo vulgar es una enfermedad ampollar autoinmune que se caracteriza por presentarse en mucosas y en la piel en zonas de roce o traumatismo. Es una variedad muy poco frecuente de la enfermedad, pero es mortal para el individuo si no se trata a tiempo. Se presenta con mayor frecuencia en mujeres entre la cuarta y sexta década de la vida. Se presentó a la consulta un paciente de sexo masculino de 35 años de edad con lesiones ampollares en la boca que le difi cultaban llevar a cabosus actividades cotidianas; las lesiones se extendieron hacia el tórax, por lo que acudió al hospital. El diagnóstico se estableció mediante una biopsia, que fue enviada al Laboratorio de Anatomía Patológica de la Facultad de Odontología de la Universidad Nacional del Nordeste, en Argentina. Para su tratamiento se prescribieron corticosteroides tópicosy sistémicos, iniciando con altas dosis y posteriormente se disminuyeron en la etapa de mantenimiento. El pronóstico fue bueno y el paciente en dos semanas fue recuperando su salud bucal...


Subject(s)
Humans , Male , Adult , Oral Manifestations , Pemphigus/classification , Pemphigus/diagnosis , Skin Manifestations , Argentina , Clinical Diagnosis , Adrenal Cortex Hormones/therapeutic use , Prognosis , Schools, Dental
15.
Rev. Salusvita (Online) ; 35(1): 95-100, 2016. ilus
Article in Portuguese | LILACS | ID: lil-788583

ABSTRACT

Relatamos um caso de pênfigo vulgar diagnosticado precocemente na consulta odontológica. Indivíduo de 42 anos apresenta-se com lesões bolhosas na mucosa bucal, com tempo de evolução aproximado de quatro meses. O laudo da biopsia incisional foi compatível com pênfigo vulgar. Imediatamente foi encaminhado para realização de exames complementares num centro especializado e, iniciou o tratamento. Geralmente, as lesões em mucosa oral precedem a aquelas em pele, dai a importância do cirurgião dentista no diagnóstico precoce desta doença potencialmente mortal.


We report a case of pemphigus vulgaris diagnosed early in a dental appointment. Male, 42 years-old, presented with bullous lesions in the oral mucosa, which developed within the last four months. The report of incisional biopsy was consistent with pemphigus vulgaris. He was immediately referred to further tests in a specialized center and started treatment. Generally, lesions in the oral mucosa precede those in the skin, hence the importance of the participation of the dentist in the early diagnosis of this potentially deadly disease.


Subject(s)
Humans , Male , Adult , Pemphigus/diagnosis , Early Diagnosis
16.
Rev. cuba. estomatol ; 52(3): 268-275, jul.-set. 2015. tab
Article in English | LILACS | ID: lil-765763

ABSTRACT

Introduction: pemphigus vulgaris is an autoimmune mucocutaneous disease, which presents at clinical examination as blisters, erosions and ulcerations of the skin and mucous membranes. Objective: the aim of this study was to review 12 cases of pemphigus vulgaris diagnosed at the Stomatology Department of São Lucas Hospital, Pontifical Catholic University of Rio Grande do Sul. Methods: the medical charts were analyzed considering patients age and sex; anatomic site and clinical aspect of the lesions; as well as symptoms, duration and clinical course of the disease. Results: the mean age of the patients was 46.7 years; most of cases occurred in females (75.0 percent); buccal mucosa was the site most frequently affected (83.3 percent), and 25 percent of cases showed extraoral manifestations. Erosion/ulceration was the most prevalent clinical appearance (83.3 percent), and pain occurred in 100 percent of cases. The mean time of development of the lesions reported by the time of the diagnosis was 12.3 months, and the mean time of follow-up was 2 years. All patients received systemic therapy with prednisone, meanwhile in 58.3 percent of cases it was associated with topic therapy and in 25 percent, with systemic immunosuppressive therapy. One case (8.3 percent) remained in remission regardless of the treatment withdrawal. Conclusion: Pemphigus vulgaris is a disease with important morbidity, which requires the attention of the dentist as a way to achieve early diagnosis(AU)


Introducción: el pénfigo vulgar es una enfermedad autoinmune mucocutánea, que presenta al examen clínico ampollas, erosiones y ulceraciones de la piel y de las membranas mucosas. Objetivo: presentar una revisión de 12 casos de pénfigo vulgar diagnosticados en el Departamento de Estomatología del Hospital São Lucas - Pontifical Catholic University of Rio Grande do Sul. Métodos: los registros médicos fueron evaluados por edad y sexo de los pacientes; el sitio anatómico y el aspecto clínico de las lesiones; así como los síntomas, duración y el curso clínico de la enfermedad. Resutados: la edad media fue 46,7 años y la mayoría de los casos fueron mujeres (75,0 por ciento). La mucosa bucal fue el sitio más frecuentemente afectado (83,3 por ciento) y el 25 por ciento de los pacientes presentaron manifestaciones extraorales de la enfermedad. La presentación clínica ulcerada fue la más frecuente (83,3 por ciento) y el dolor estaba presente en el 100 por ciento de los casos. La duración media de las lesiones en el diagnostico fue de 12,3 meses y el tiempo medio del seguimiento fue de 2 años. Todos los pacientes recibieron la terapia sistémica con prednisona. En el 58,3 por ciento de los casos hubo una asociación con terapia tópica y en el 25 por ciento con terapia inmunosupresora. Uno de los casos (8,3 por ciento) permaneció en remisión después de la interrupción de la terapia. Conclusión: el pénfigo vulgar es una enfermedad con una morbilidad importante, que requiere la atención del dentista como una manera de lograr el diagnóstico temprano(AU)


Subject(s)
Humans , Female , Middle Aged , Pemphigus/diagnosis , Immunosuppressive Agents/therapeutic use , Mouth Diseases/epidemiology , Mouth Mucosa/injuries , Medical Records/statistics & numerical data
17.
Rev. ADM ; 72(4): 218-220, jul.-ago. 2015. ilus
Article in Spanish | LILACS | ID: lil-775323

ABSTRACT

El pénfigo vulgar es el tipo más común de un grupo padecimientos crónicos autoinmunes identificados por la presencia de lesiones ampulosas situadas en las mucosas y piel. El pénfigo vulgar oral (PVO) se caracteriza por la presencia de ampollas localizadas en las encías, paladar blando, carrillos, pero cualquier sitio de la cavidad oral puede ser afectado. Estas lesiones se presentan primero en la cavidad oral y meses después en la piel, por lo que su diagnóstico temprano y oportuno es vital para el pronóstico. El propósito de este trabajo es presentar esta condición clínica en una persona adulta mayor.


Pemphigus vulgaris is the most common of a group of chronic autoim-mune conditions characterized by the presence of mucosal and dermal blisters. In the case of oral pemphigus vulgaris (OPV), these are typi-cally found on the gums, soft palate, and cheeks, though anywhere in the oral cavity can be affected. These lesions appear first in the oral cavity and then, months later, on the skin. Therefore, early diagnosis is crucial for prognosis. The aim of this paper is to present a case report of this condition in an older adul.


Subject(s)
Humans , Female , Aged , Mouth Diseases/classification , Pemphigus/diagnosis , Pemphigus/drug therapy , Prognosis , Prednisolone/therapeutic use , Treatment Outcome
20.
Article in English | IMSEAR | ID: sea-159379

ABSTRACT

Pemphigus vulgaris is an autoimmune mucocutaneous disorder that affects the skin and other mucous membranes of which the intra-oral lesions are first to appear. The occurrence of this disease is very few in the general population. Nevertheless, pemphigus vulgaris is a critical condition because if untreated, it often results in patient’s death. This disease is characterized by the production of autoantibodies against intercellular bridges. Most of the patients are misdiagnosed. Hence, it is necessary for the dental professionals to be aware with the clinical manifestations of this disease to ensure early diagnosis and treatment. Here, we present a case of 57-year-old female patient, which was diagnosed as pemphigus vulgaris.


Subject(s)
Female , Fluorescent Antibody Technique/methods , Humans , Middle Aged , Mouth Diseases , Pemphigus/cytology , Pemphigus/diagnosis , Pemphigus/therapy
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