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Pênfigo Vulgar com Envolvimento Anal
Vieira,Ana Margarida; Cardoso,Cláudia; Gamito,élia; Alves,Ana Luísa; Oliveira,Ana Paula;
Jornal Português de Gastrenterologia , 2011,
Abstract: introduction: anal involvement in patients with pemphigus vulgaris has rarely been reported. we report a case of a 76-year-old woman with muco-cutaneous pemphigus vulgaris on systemic corticotherapy who started with hematochezia and anal pain associated with constipation and difficult defecation. flexible sigmoidoscopy was normal but proctologic examination revealed skin tags with multiple erosions. after local corticotherapy and normalization of bowel movements, healing of anal lesions was seen.
PéNFIGO VULGAR: CRITERIOS ACTUALES Pemphigus Vulgaris: Current approaches
Isis Bárbara Herrera López,Josefa Miranda Tarragó
Revista Habanera de Ciencias M??dicas , 2009,
Abstract: Se realizó una revisión bibliográfica sobre pénfigo vulgar, utilizando varias bases de datos como: LIS, MEDLINE, COCHRANE, LILACS, SeCimed; con el objetivo de actualizar la información sobre esta patología, en cuanto a: prevalencia, signos y síntomas más frecuentes, morfología, métodos diagnósticos que se emplean y tratamiento. Esta enfermedad cutáneo-mucosa, no es muy frecuente en nuestro país (0.1 %-0.5%); sin embargo, es la forma más habitual y grave de los pénfigos. Se presenta con mayor frecuencia en la cuarta y sexta década de la vida, siendo extra a en ni os. No tiene predilección ni por el sexo ni por la raza, su etiología no está definida, aunque se asocia con trastornos inmunológicos. Se caracteriza por la aparición de ampollas relativamente tensas, en piel y mucosas, las cuales posteriormente se rompen y forman erosiones de superficies maceradas, recubiertas con material blanquecino y muy dolorosas. En las mucosas se pueden extender a labios, faringe y laringe, dificultando la ingestión de alimentos, haciendo que los pacientes se debiliten, y junto con la pérdida de electrolitos a través de las lesiones de piel, puedan llevarlo a la muerte. Lo que motivó esta revisión fue que generalmente, se presenta primero en la boca, por lo que es de suma importancia que el estomatólogo esté familiarizado con la enfermedad, a fin de diagnosticarla y tratarla precozmente, haciendo que el tratamiento sea más sencillo y, por lo tanto, menos doloroso y molesto al paciente. It was carried out a literature review on vulgar pemphigus, using several databases like: LIS, MEDLINE, COCHRANE, LILACS, SECIMED; with objective of modernizing information on this pathology, as for: prevalence, signs and more frequent symptoms, morphology, diagnoses methods that are used and treatment. This mucous coetaneous illness isn′t very frequent in our country (0.1% -0.5%) however it′s most usual and serious pemphigus variety. It′s presented with more frequency between fourth and sixth decade of life, being uncommon in children. It haven't predilection neither for genre neither for race, their etiology is not defined, although it associates with immunologic dysfunctions. It′s characterized by appearance of relatively tense bladders, in skin and mucous, forming erosions of macerated surfaces, whitish covered and very painful material later. In mucous ones they can extend to lips, pharynx and larynx, hindering ingestion of foods, making patients to weaken, and together electrolytes through the skin lesions, they can take it to death. What motivated this revision was that generally, it
Pénfigo vulgar importancia para el odontólogo en el diagnóstico Pemphigus vulgaris - importance of the dentist in its diagnosis
Luciana Estevam Simonato,Ana Carolina Prado Ribeiro,Alan Roger dos Santos Silva,Ana Maria Pires Soubhia
Acta Odontológica Venezolana , 2008,
Abstract: El pénfigo vulgar es una enfermedad vesiculo ampollar crónica y grave que compromete piel y mucosas. La presencia de anticuerpos antidesmosómicos resulta en la disolución de la sustancia cementante intercelular para la destrucción de los desmosomas que mantienen unidas a las células epiteliales generando una separación intraepitelial característica que ocurre luego arriba de la camada de células basales. El pénfigo vulgar es una condición de revelancia odontológica que si no estratada puede resultar en la muerte del paciente. El compromiso de la cavidad bucal en la mayoria de los casos aliado a la importancia del diagnóstico precoz para el pronóstico y tratamiento, refuerzan la necesidad del conocimiento de esa condición por el odontólogo. Pemphigus vulgaris is a chronic and serious auto-imune vesiculo-bullous disease that affects skin and mucous membrane. Antidesmosomal antibodies dissolve intercellular substance that keeps epithelial cells together, resulting in an intraepithelial blister just above basal epithelial layer. Without treatment, pemphigus vulgaris leads patient to die. The fact that this disorder presents oral manifestation in most cases, in addiction to the relevance of a precocious diagnosis to the success of the treatment and prognosis support the importance of about the knowledge of this disease to dentist's notice.
Inmunopatogenia del pénfigo vulgar y el pénfigo foliáceo = Immunopathogenesis of pemphigus vulgaris and pemphigus foliaceus
Valencia Ocampo, óscar Jairo,Velásquez Lopera, Margarita María
Iatreia , 2011,
Abstract: El pénfigo vulgar y el pénfigo foliáceo son enfermedades ampollosas autoinmunes mediadas por autoanticuerpos dirigidos contra proteínas de los desmosomas, las desmogleínas 1 y 3. Están asociadas con moléculas del complejo mayor de histocompatibilidad (HLA) que por su estructura tienen la capacidad de presentar péptidos antigénicos de las desmogleínas. En los individuos afectados se han descrito la presencia de linfocitos T y B autorreactivos y alteraciones en la regulación del sistema inmune con desequilibrio de las respuestas Th1/Th2. No se conocen con precisión los mecanismos de da o pero la investigación actual indica que los anticuerpos tienen un papel patogénico, inician diferentes cascadas de se alización que provocan la acantólisis y apoptosis de los queratinocitos. El conocimiento de la inmunopatogenia de las enfermedades ampollosas autoinmunes ha permitido el desarrollo y la puesta en práctica de nuevas alternativas terapéuticas.
Prevalence of thyroid autoimmunity in patients with pemphigus vulgaris Prevalencia de autoinmunidad tiroidea en pacientes con pénfigo vulgar  [cached]
Fabián Pitoia,Daniel Moncet,Roberto Glorio,Adriana Graciela Díaz
Medicina (Buenos Aires) , 2005,
Abstract: Among bullous diseases, pemphigus vulgaris (PV) is a classical variety of this type of skin disorders. To establish the real prevalence of thyroid abnormalities in such a disease, a prospective study was developed. For this reason, thyroid evaluation was performed in 15 consecutive patients who attended the Dermatology Clinic for PV and in a group of 15 healthy volunteers (Control Group) matched by age and gender. Thyroid function was evaluated by measuring T3, T4 and TSH. The presence or absence of goiter was searched by palpation, while thyroid autoimmunity was investigated through the assay of thyroperoxidase antibodies (TPO-Ab). In each group there were 9 women and 6 men, aging 25-65 years (mean = 48.3 y) in the PV Group, and 25-69 years (mean = 45.4 y) in the Control Group. It was found that 7 patients (46.6%) of the PV Group and 1 subject (6.7%) of the Control Group (p < 0.015) disclosed thyroidal alterations. Positive titers of TPO-Ab were observed in 6 patients with PV and in one volunteer. Goiter and subclinical hypothyroidism were found in one PV patient with negative TPO-Ab. Out of the total 7 cases with positive TPO-Ab, only a PV patient had an overt Hashimoto’s thyroiditis. All other cases had only the presence of thyroid auto-antibodies without clinical evidences of chronic thyroiditis. It is concluded that PV is highly associated with primary thyroid disorders, mainly with positive titers of TPO-Ab, although most patients do not present overt clinical thyroid disease. El pénfigo vulgar (PV) es una enfermedad ampollar clásica de etiología autoinmune que se caracteriza por la presencia de lesiones intraepiteliales. Para establecer la prevalencia de anormalidades tiroideas en el PV, realizamos un estudio prospectivo en 15 pacientes consecutivos que consultaron a la División Dermatología debido a PV y en un grupo de 15 voluntarios sanos (Grupo Control). La función tiroidea se evaluó a través de la medición de T3, T4 y TSH y la presencia de bocio se determinó por medio de la palpación tiroidea. La autoinmunidad se investigó usando un ensayo IRMA para la medición de anticuerpos antitiroperoxidasa (ATPO). En cada grupo había 9 mujeres y 6 hombres que fueron apareados por edad y sexo, con edades comprendidas entre 25 y 65 a os (promedio 48.2 a os) en el grupo PV, y entre 25 y 69 a os (promedio 45.4 a os) en el grupo control. Se encontró que 7 pacientes (46.6%) del grupo PV y uno (6.6%) del grupo control presentaron alteraciones tiroideas, (p<0.015). La presencia de ATPO positivos se observó en 6 pacientes con PV y en un voluntario del grupo contr
Pénfigo vulgar bucal precedido de lesiones Cutáneas: reporte de un caso ORAL PEMPHIGUS VULGARIS PRECEDING CUTANEOUS LESIONS: CASE REPORT
Janet Lazarde L,María T Campos Ch
Acta Odontológica Venezolana , 2004,
Abstract: El Pénfigo Vulgar forma parte de un grupo de enfermedades que se manifiestan por ampollas en piel y membranas mucosas, apareciendo con muy alta frecuencia en la mucosa bucal. Generalmente, se presenta primero en la boca, por lo que es de suma importancia que el odontólogo esté familiarizado con la enfermedad con el fin de diagnosticarla y tratarla precozmente, previniendo que las lesiones cutáneas aparezcan y haciendo que el tratamiento sea más sencillo. Se presenta una revisión bibliográfica y se reporta el caso de una paciente que asistió a la consulta del Servicio de Clínica Estomatológica de la Facultad de Odontología, U.C.V., con manifestaciones bucales de pénfigo vulgar. El objetivo principal de este artículo es ofrecer al odontólogo una información detallada de esta patología, ya que es necesario que esté capacitado para diagnosticarla y tratarla en conjunto con el Patólogo Clínico. Pemphigus Vulgaris is a disease that appears as blisters in the skin and mucous membranes, showing in a high rate at bucal mucous. First, usually develops in the mouth and it’s very important that the dentist is aware of the illness, so that can be previously diagnose and treat it on an early stage; preventing to show this cutaneous injury and making the cure more simple. In a bibliographic review, is reported a case of a patient that assisted at the Stomatologic Clinic service in Dentist Faculty, U.C.V. with bucal Pemphigus Vulgaris manifestations. The purpose of this article is to offer the dentist detail information of this pathology because it seems necessary that he is trained in diagnosis and treatment linked with Clinical Pathologist Doctor.
Ampolla conjuntival en contexto de pénfigo vulgar: a propósito de un caso Conjuntival blistering associated with pemphigus vulgaris: report of a case
C Bianciotto,JM Herreras Cantalapiedra,MA Saornil álvarez,MC Méndez Díaz
Archivos de la Sociedad Espa?ola de Oftalmología , 2005,
Abstract: Caso clínico: Un paciente con una ampolla conjuntival fue diagnosticado de pénfigo vulgar mediante técnicas de inmunofluorescencia en biopsia conjuntival. Discusión: El pénfigo vulgar es una enfermedad autoinmune grave pero poco frecuente que produce ampollas en piel y membranas mucosas. La afectación ocular es poco común e incluye conjuntivitis y erosiones palpebrales. Ampollas y erosiones conjuntivales asociadas no han sido aún descritas en la literatura. Se llega al diagnóstico mediante técnicas de inmunofluorescencia directa realizada en biopsias de tejido afectado. La mortalidad es elevada si no es diagnosticada y tratada adecuadamente. Clinical case: A patient with a conjunctival blister was diagnosed with pemphigus vulgaris by immunofluorescence tests performed on a conjunctival biopsy. Discussion: Pemphigus vulgaris is an uncommon but serious autoimmune disease that produces blisters of the skin and mucous membranes. Ocular findings are rare, but include conjunctivitis and marginal eyelid erosions. Conjunctival blisters and erosions related to this condition have not been previously reported in the literature. This diagnosis can be made through direct immunofluorescence tests performed on biopsy samples of affected tissue. Unless the condition is properly diagnosed and treated, it has a high mortality.
Pemphigus vulgaris  [PDF]
Ajay Prakash P,Geetha P,Surekha R,Madhusudhan Rao
Indian Journal of Dental Advancements , 2009,
Abstract: Pemphigus vulgaris is a severe autoimmune disorder of the skin and mucous membranes that is characterised by the formation of bulla. Oral mucosa plays an important role in the natural history of Pemphigus vulgaris. In approximately 50-70 percent of patients, the disease starts in the oral cavity and the lesions in oral mucosa precede the cutaneous lesions by several months. Very rarely lesions remain restricted to oral mucosa for prolonged period. We here in report one such case recently seen by us, where oral lesions are predominant with little or no skin lesions.
Anal Involvement in Pemphigus Vularis  [PDF]
Somayeh Khezri,Hamid-Reza Mahmoudi,Seyedeh Nina Masoom,Maryam Daneshpazhooh,Kamran Balighi,S. Hamed Hosseini,Cheyda Chams-Davatchi
Autoimmune Diseases , 2013, DOI: 10.1155/2013/609181
Abstract: Background. Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucosa. Anal mucosa may be involved in PV, but the frequency and clinical profile are not fully ascertained. Objective. The aim was to investigate the involvement of the anal area in newly diagnosed PV patients. Patients and Methods. A total of 168 consecutive newly diagnosed PV patients were enrolled. Anal symptoms and signs, involvement of other body sites, and severity of disease were recorded. Results. A total of 47 out of 168 patients (27.9%) had involvement of the anal area. Anal involvement was significantly associated with PV lesions in ophthalmic ( ), nasal ( ), and genital mucosa ( ) but not the oral cavity ( ). There was a significant association between number of involved mucosal sites and anal involvement ( ). Anal involvement was associated with oral severity ( ). Constipation was the most frequent symptom (73.8%) followed by pain on defecation (50%). Seventeen patients (36%) were symptom-free. Erosion was the most frequent sign (91.5%). Conclusion. Anal involvement in PV seems to be more frequent than previously assumed. Routine anal examination is recommended even in asymptomatic patients as anal involvement appears to correlate with the severity of PV. 1. Introduction Pemphigus vulgaris (PV) is a rare, autoimmune, potentially fatal mucocutaneous bullous disease in which pathogenic autoantibodies are directed against the keratinocyte cell surface molecules desmoglein 3 (Dsg3) and to a lesser extent Dsg1 [1]. The incidence of this disease varies from 0.16 to 1.62 cases per 100,000 with increased incidence in Jews, Indians, and middle easterners [2]. PV is characterized by bullae that typically begin in the oral cavity and may spread to involve the skin. Other mucosal surfaces including conjunctiva, nasal mucosa, pharynx, larynx, epiglottis, esophagus, cervix, vagina, and penile mucosa may also be affected in the course of disease [3–8]. Anal involvement may also be seen in PV but its frequency and clinical profile are not fully ascertained yet [9–12]. The aim of this study was to investigate the involvement of the anal area in newly diagnosed PV patients presenting to the Autoimmune Bullous Diseases Research Center (ABDRC), Tehran, Iran, during a 15-month period. 2. Patients and Methods This prospective study included 168 consecutive patients newly diagnosed with PV, attending the ABDRC, between October 2009 and January 2011. The diagnosis of PV was based on the presence of clinical features of the disease, including mucocutaneous bullae and erosions
Transition of pemphigus vulgaris to pemphigus foliaceus
Mendiratta Vibhu,Sarkar Rashmi,Sharma R,Korann R
Indian Journal of Dermatology, Venereology and Leprology , 2000,
Abstract: We report a case of pemphigus vulgaris in a 46-year-old woman who presented with scaly and crusted erosions over erythematous base associated with itching and burning mainly over the chest, submammary folds, face, abdomen and the back. Histopathology was consistent with pemphigus foliaceus. The patient responded to oral prednisolone (60 mg/day). The above findings suggest that pemphigus vulgaris showed a transition to the pemphigus foliaceus variant.
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