Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

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.

Oxidative stress in patients with endemic pemphigus foliaceus and healthy subjects with anti-desmoglein 1 antibodies

Abstract: Background: Previous studies have shown oxidative stress in pemphigus vulgaris and pemphigus foliaceus, nevertheless, it remains unknown whether a similar response is characteristic of endemic pemphigus foliaceus in Peru. Objectives: To determine the oxidative stress response in endemic pemphigus foliaceus patients and subjects with positive for anti-desmoglein1 antibodies (anti-dsg1) from endemic areas of Peru. Subjects and Methods: This is a cross-sectional study. The study population included 21 patients with Endemic Pemphigus foliaceus and 12 healthy subjects with anti-dsg1 antibodies from the Peruvian Amazon (Ucayali), as well as 30 healthy control subjects. Malondialdehyde, an indicator of lipid peroxidation by free radicals, was measured in serum. Results: We collected 21 cases of endemic pemphigus foliaceus, 15 of them with active chronic disease and 6 in clinical remission. Serum malondialdehyde values in patients with chronic active evolution and healthy subjects with anti-dsg1 antibodies were statistically higher than those of healthy controls (p<0.001). There was no significant difference between serum values of localized and generalized clinical forms. Study limitations: The main limitation of this present study is the small number of patients with endemic pemphigus and healthy subjects positive for desmoglein 1 antibodies. Conclusions: The increased serum levels of malondialdehyde in patients with chronic active endemic pemphigus foliaceus and healthy subjects from endemic areas with anti-dsg1 antibodies may suggest a contribution of systemic lipid peroxidation in the pathogenesis of endemic pemphigus foliaceus.

Hepatitis C and hepatitis B virus infections in the etiopathogenesis of pemphigus

BACKGROUND: Previous studies have shown that some viral infections may be triggers for autoimmune diseases. The role of viral infections in the etiopathogenesis of pemphigus has also been investigated. OBJECTIVES: To investigate the relationship between pemphigus and the hepatitis B and C virus infections. METHODS: This retrospective study included 62 patients with pemphigus and 50 healthy controls of matching ages and genders. The control group included relatives who accompanied the orthopedic patients to the hospital but had no history of systemic and/or autoimmune diseases. RESULTS: The group of patients with pemphigus was composed of 43 (69.3%) females and 19 (29.7%) males, and the mean age was 48.08 ± 15.38. The hepatitis C virus antibody was negative in all of the patients. Hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), or both, were studied in 44 of the 62 patients. Two (4.3%) of these patients were positive for HBsAg and found to have HBV infection. Fourteen of them were HBsAb positive. Two (4%) of the 50 control subjects showed hepatitis C virus antibody positivity, while only 1 (2%) patient with pemphigus displayed HBsAg positivity. There was no statistically significant difference between the two groups for hepatitis B and C virus infections. CONCLUSIONS: This study does not support an association between pemphigus and hepatitis B and/or C virus infections. .

IgG/IgA pemphigus reactive with desmoglein 1 with additional undetermined reactivity with epidermal basement membrane zone.

IgG/IgA pemphigus is an extremely rare subset of pemphigus, showing anti-keratinocyte cell surface antibodies of both IgG and IgA classes. Herein, we describe a unique case of IgG/IgA pemphigus with clinical features of edematous erythema and peripheral vesiculopustules. Histopathology showed the presence of subcorneal pustules and acantholytic blisters in the mid-epidermis with neutrophilic infiltration and eosinophilic spongiosis. Direct immunofluorescence of perilesional skin showed both IgG and IgA deposits to keratinocyte cell surfaces and unusual granular deposits of IgG, IgM, and C3 along basement membrane zone. On enzyme linked immunosorbent assay , the auto-antibodies were found to be reactive to desmoglein 1 antigen. Various clinical, histopathological, and immunological findings in our case overlapped with the features of IgA pemphigus, pemphigus herpetiformis, and pemphigus foliaceus. These findings indicate that IgG/IgA pemphigus may be a transitional form between IgA pemphigus and pemphigus herpetiformis, and thus provides insight into the pathogenicity of this rare disorder.

Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.

Evaluación inmunopatológica del pénfigo foliáceo endémico luego de seis meses de tratamiento: seguimiento de cinco casos de la Amazonía peruana / Evaluation of the immunopathologic response in patiens with endemic pemphigus foliaceus after six months of systemic corticosteroid therapy

Objetivo. Describir la respuesta inmunopatológica de cinco pacientes peruanos con pénfigo foliáceo endémico antes y seis meses después de la terapia con corticosteroides sistémicos. Material y métodos. Estudio descriptivo de tipo serie de casos. Los pacientes fueron sometidos a examen físico, se obtuvodatos cínicos y, luego, una muestra de sangre, a la que se le realizó inmunofluorescencia indirecta (IFI). Se usó como sustratos esófago de mono y piel humana normal, Elisa IgM antidesmogleína 1, Elisa IgG antidesmogleína 1 (total y subclases), inmunoprecipitación (IP) antidesmogleína 1 y antidesmogleína 3. Los pacientes recibieron tratamiento con corticosteroides sistémicos por seis meses obteniéndose nuevamente una muestra de sangre para estudiar su evolución inmunopatológica. Resultados. Al examen físico inicial tres pacientes presentaban la forma clínica generalizada y dos, la localizada; a los seis meses de tratamiento solo un paciente presentaba lesiones activas debido a que su tratamiento había sido irregular. La IgManti-DSG-1 no mostró variaciones importantes manteniéndose positiva en un paciente y haciéndose positiva en un paciente inicialmente negativo. El Elisa para anticuerpos IgG4 se redujo notablemente en todos los pacientes incluidos los que tenían tratamiento irregular. De los dos pacientes que presentaban anticuerpos anti-DSG-3 (Elisa) uno redujo sus valores índices y elotro se hizo negativo; mientras que un paciente inicialmente negativo desarrolló al final del tratamiento anti-DSG-3. Conclusión. La respuesta antidesmogleína 1 IgG total e IgG4 así como la respuesta antidesmogleína 3 muestran reducción de sus valores índices con tendencia a la negativización luego de seis meses de tratamiento lo cual es evidenciado también con la IFI. La respuesta IgM antidesmogleína 1 no mostró variaciones importantes a pesar del tratamiento.

Aim. To describe the immunopathologic response in five patients with endemic pemphigus foliaceus (EPF) before and after six months of therapy with systemic corticosteroids. Material and methods. Case series study. Each patient was examined by a dermatologist who gathered the clinical information. A serum sample was obtained for indirect immunofluorescence (IFI) with monkey esophagous (ME) and normal human skin (NHS) assubstrates. Also, Elisa for IgM and IgG (total and subclasses) antidesmoglein 1 antibodies and immunoprecipitation (IP) for antidesmoglein 1 and 3 were carried out. All the patients received treatment with systemic corticosteroids for six months and blood serum was drawn after wards to assess the immunopathologicresponse. Results. On physical exam, 3 patients had the generalized form ofEPF, 2 patients had the localized form and one patient had someactive lesions because he was not compliant with the therapy.Anti-IgM desmoglein 1 values were decreased in 4 patients buta couple of the values remained unchanged or even increased because they were not completely compliant with the treatment.IgG4 desmoglein 1 antibodies were notably decreased in all the patients. A couple of patients who had antibodies against desmoglein 3 decreased, being one of them negative. One of the patients was initially negative for antibodies against desmoglein3 but later on, he developed anti desmoglein 3 antibodies.Conclusion. The immunological response against IgG and IgG4desmoglein 1 as well as against desmoglein 3 were reduced after the treatment and showed a tendency to become negative. This was correlated with IFI values as well. IgM desmoglein antibodies didnot show important variations despite of the corticosteroid treatment.

Analysis of the reactivity of indirect immunofluorescence in patients with pemphigus foliaceus and pemphigus vulgaris using rat bladder epithelium as a substrate

OBJECTIVES: To evaluate the reactivity of indirect immunofluorescence using rat bladder epithelium as a substrate in patients with pemphigus foliaceus and pemphigus vulgaris from the Department of Dermatology, University of São Paulo Medical School, Brazil. METHODS: Thirty-two patients (8 male and 24 female) from the Department of Dermatology, University of São Paulo Medical School, were selected. Three had mucosal pemphigus vulgaris, 20 had mucocutaneous pemphigus vulgaris, and 9 had pemphigus foliaceus. Patients’ sera were tested by indirect immunofluorescence performed on human foreskin and rat bladder epithelium and by ELISA assays utilizing baculovirus-expressed recombinant desmoglein 3 and desmoglein 1. RESULTS: No patients with mucosal pemphigus vulgaris, 5 of 20 patients with mucocutaneous pemphigus vulgaris (25 percent) and 4 of 9 patients with pemphigus foliaceus (44 percent) had positive indirect immunofluorescence using rat bladder epithelium as a substrate. CONCLUSION: Indirect immunofluorescence using rat bladder epithelium as a substrate is recommended whenever a diagnosis of paraneoplastic pemphigus is considered. The identification of a subset of pemphigus foliaceus and pemphigus vulgaris patients that recognizes desmoplakins by this laboratory tool is critical to avoid the misdiagnosis of paraneoplastic pemphigus.

Pemphigus vulgaris during pregnancy - a case report

Pemphigus vulgaris [PV] is an uncommon immune-mediated bullous dermatosis which is very rare during pregnancy. Its management during pregnancy is a challenge and sometimes very difficult. Only few cases have been reported in literature so far. The disease may be associated with adverse fetal outcomes such as prematurity and fetal death. The neonate can develop transient skin lesions. We present a case of a patient who conceived during the active phase of PV required high doses of corticosteroids and delivered a preterm appropriate for gestation age newborn

Gestante con pénfigo foliáceo endémico y producto libre de enfermedad: Caso clínico / Endemic pemphigus foliaceus in a pregnant woman: Report of one case

Endemic pemphigus foliaceus (EPF) in an autoinmune skin disease present in areas of the Amazonia. We report a 36 year-old woman who presented EPF at 17 weeks of pregnancy. At 29 weeks, she started antimicrobial treatment and steroids. At the moment of delivery, the disease was in remission and cutaneous lesions were not seen in the neonate. Indirect immunofluorescence titers of total IgG in the mother and in the neonate were negative. Sixteen months later, IgG titers in the offspring were 1/20 and remained negative in the mother, who was on low doses of oral corticosteroids (Rev Méd Chile 2009; 137: 1205-8).

Dermatoses bolhosas auto-imunes / Autoimmune bullous dermatoses

Dermatoses bolhosas autoimunes são doenças cuja manifestação cutânea primária e fundamental consiste em vesículas e bolhas. Classificam-se conforme a localização da bolha, em intraepidérmica e subepidérmica. Os pacientes produzem autoanticorpos contra estruturas específicas da pele detectáveis por técnicas de imunofluorescência, immunobloting e Elisa. Os recentes avanços da biologia molecular e celular têm permitido conhecer esses autoantígenos, contra os quais os pacientes se sensibilizam e que estão localizados na epiderme ou na junção dermoepidérmica. São doenças de baixa incidência, porém de elevada morbidade e por vezes letais. O objetivo deste trabalho é revisar e descrever os progressos nos conhecimentos de quatro doenças vésico-bolhosas autoimunes: pênfigo foliáceo endêmico (fogo selvagem), pênfigo vulgar, penfigóide bolhoso e dermatite herpetiforme.

Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire), pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis.

Pénfigo foliáceo con viraje a pénfigo vulgar / Pemphigus foliaceus to pemphigus vulgaris shift to

El pénfigo es un grupo de enfermedades autoinmunes adquiridas, caracterizado por formación de ampollas en la piel y las mucosas, debido a la pérdida de cohesión entre los queratinocitos. Puede dividirse en cuatro tipos principales: foliáceo, vulgar, paraneoplásico y por IgA. El viraje de un tipo de pénfigo a otro es infrecuente y su fisiopatogenia poco clara, aunque se has postulado diversas hipótesis que involucran a dos fenómenos inmulógicos, el mimetismo molecular y la amplificación del epítope. A continuación se comunica el caso de un paciente de 71 años con pénfigo foliáceo, que durante el tratamiento con corticoesteroides presentó una erupción variceliforme de Kaposi por herpes virus tipo 1 y tres meses después evolucionó con el desarrollo de manifestaciones clínicas e histopatológicas características de pénfigo vulgar.

Correlation of pemphigus vulgaris antibody titers by indirect immunofluoresence with activity of disease based on pemphigus area and activity score [PAAS]

Indirect immunofluorescence [IIF] has been used to identify and measure autoantibody levels in pemphigus vulgaris but data about relationship between clinical severity of disease and antibody titers by IIF have been conflicting. We conducted this cross-sectional study to correlate the severity of oral and/or cutaneous involvement in patients with pemphigus vulgaris based on Pemphigus Area and Activity Score with IIF titers. Sixty-one new pemphigus vulgaris patients were included in this study. Human prepuce was used as substrate for IIF and assessment of disease severity was based on Pemphigus Area and Activity Score. The mean +/- SD age was 44.04 +/- 30.46 years, with a range of 18 to 79 years. IIF was positive in 56 [91.8%] patients. There was a significant relationship between total disease score and IIF titers [[P<0.001]. Also a significant relationship was found between skin score [P=0.04] and mucosal score [P=0.04] with IIF titers. Our results show that there is a significant relationship between disease activity based on Pemphigus Area and Activity Score and antibody titers by IIF. Further studies are recommended to determine the usefulness of this technique for monitoring disease

Enfermedades virales y autoinmunes en pacientes con pénfigo foliáceo endémico y su influencia en la inmunología de la enfermedad / Viral and autoimmune disease in endemic penfigus foliaceus patients and their influence over the disease immunology

Objetivo: Determinar la presencia de enfermedades virales y autoinmunes en pacientes con pénfigo foliáceo endémico (PFE) y su influencia sobre la inmunología de la enfermedad. Material y métodos: Estudio prospectivo realizado en la provincia de Coronel Portillo (Ucayali-Perú) de octubre del 2001 a septiembre del 2005. La muestra estuvo constituida por 15 pacientes con PFE captados en el Hospital Regional de Pucallpa así como en comunidades y/o distritos que cumplen con las características de foco endémico (Nueva Requena, Campo Verde, Yarinacocha) a los que se les realizó inmunofluorescencia indirecta, ELISA para anticuerpos anti desmogleína 1 y anti desmogleína 3. Para confirmar el diagnóstico previo de fiebre del dengue se evaluó la presencia de anticuerpos lg G mediante ELISA de captura para virus del dengue. Resultados: Para las enfermedades virales la prevalencia fue de 26.7 por ciento, se presentó un caso de herpes simple, un caso de erupción variceliforme de Kaposi y dos casos de fiebre del dengue. Las enfermedades autoinmunes se presentaron en tres casos, dos de psoriasis vulgar y otro de vitíligo no segmentario localizado. La presencia de enfermedades virales y/o autoinmunes no influyeron significativamente en los valores índices de anticuerpos antidesmogleína 1 e inmunofluorescencia indirecta. Conclusiones: Existe una baja frecuencia de enfermedades virales y/o autoinmunes en pacientes con PFE, y cuando se presentan no influyen significativamente en la inmunología de la enfermedad.

Pénfigo foliáceo endémico en el Perú: caracterización clínica, epidemiológica e inmunopatológica / Endemic pemphigus foliaceus in Perú: clinical, epidemiological and immunopathologic characterization

Objetivo: Determinar las características clínicas, epidemiológicas e inmunopatológicas de los pacientes con pénfigo foliáceo endémico, en el Perú. Pacientes y métodos: Estudio comparativo, prospectivo y multicéntrico realizado de julio de 2002 a junio de 2006. La muestra estuvo constituida por 52 pacientes con pénfigo foliáceo endémico, captados en el Hospital Regional de Pucallpa, Hospital de Apoyo de Yarinacocha, Puesto de Salud de Pueblo Libre (Ucayali), Hospital Nacional Dos de Mayo (Lima), y mediante búsqueda activa. Se registró las características clínicas y epidemiológicas; el estudio inmunopatológico (Elisa, inmunoprecipitación e inmunofluorescencia indirecta) se realizó en 16 pacientes con pénfigo foliáceo endémico, 41 sujetos sanos de un área endémica (Pueblo Libre), 11 familiares de pacientes y 20 donantes sanos procedentes de áreas no endémicas de Pucallpa, Lima y Chachapoyas. Resultados: El pénfigo foliáceo endémico es endémico en Ucayali, Loreto, Huánuco, Amazonas, Junín y San Martín; la edad promedio de los pacientes fue 31,4 ± 18,3 años y 57,7 por ciento correspondió al sexo masculino. El 65,4 por ciento presentó la forma clínica generalizada y el resto la forma localizada. Se detectó anticuerpos antidesmogleína 1 mediante Elisa en 13/16 pacientes (81,3 por ciento) y antidesmogleína 3, en 5/16 (31,3 por ciento). Los pacientes con pénfigo foliáceo endémico presentaron anticuerpos antidesmogleína 1 de tipo IgG4, IgG1 e IgG2. Los sujetos sanos del foco endémico de Pueblo Libre y los familiares de los pacientes con pénfigo foliáceo endémico también desarrollaron anticuerpos antidesmogleína 1 y 3. Conclusiones: El pénfigo foliáceo endémico, en el Perú, es una enfermedad que se presenta en adolescentes y adultos jóvenes, siendo la forma clínica predominante la generalizada...

Objective: To determine clinical, epidemiological and immunopathologic characteristics of endemic pemphigus foliaceus in Peru. Patients and methods: Prospective and multicentric study from July 2002 through June 2006. The sample consisted in 52 patients with endemic pemphigus foliaceus seen in the following centers: Pucallpa Regional Hospital, Yarinacocha Hospital, Pueblo Libre Health Center (Ucayali), Hospital Nacional Dos de Mayo (Lima) and by active research. Clinical and epidemiological characteristics were registered; immunopathologic study (Elisa, immunoprecipitation, and indirect immunoflourescence tests) was performed in 16 patients with endemic pemphigus foliaceus, in 41 healthy individuals of an endemic area (Pueblo Libre community), 11 patients’ relatives and in 20 healthy donors from non endemic areas of Pucallpa, Lima and Chachapoyas.Results: endemic pemphigus foliaceus is endemic in Ucayali, Loreto. Huanuco, Amazonas, Junin and San Martin. Patients’ mean age was 31,4 ± 18,3 years and 57,7% were male. The generalized presentation occurred in 65,4% of patients. Anti-desmoglein 1 antibodies were detected by Elisa in 13 of 16 patients (81,3%) and anti-desmoglein 3 in 5 of 16 (31,3%). Patients with endemic pemphigus foliaceus presented IgG4, IgG1 and IgG2 antidesmoglein antibodies. Moreover healthy subjects of the endemic focus of the Pueblo Libre community and the patients’ relatives developed antibodies against desmoglein 1 and 3.Conclusions: In Peru, endemic pemphigus foliaceus is a disease that occurs in adolescents and young adults; the predominant clinical presentation is generalized. The immunologic profile of patients with endemic pemphigus foliaceus is predominantly IgG4. Healthy subjects of endemic areas and patients’ relatives developed anti-desmoglein 1 and 3 antibodies.

Prevalencia de disfunción tiroidea primaria en pacientes con pénfigo vulgar / Prevalence of primary thyroid disorders in patients with pemphigus vulgaris

Introducción. Los pénfigos representan un grupo de enfermedades autoinmunes caracterizadas por la formación de ampollas. Dentro de este grupo, el pénfigo vulgar es la forma clínica más frecuente. Por otro lado, la patología tiroidea tiene elevada prevalencia en la práctica médica y está asociada con un amplio rango de trastornos cutáneos. Hay publicaciones aisladas de la asociación entre patología tiroidea y pénfigo vulgar. Por esta razón, se decidió establecer la prevalencia de disfunción tiroidea primaria y autoinmunidad tiroidea en pacientes con pénfigo vulgar. Objetivos. (i) Determinar la prevalencia de disfunción tiroidea primaria y autoinmunidad tiroidea en pacientes con pénfigo vulgar. (ii) Relacionar los hallazgos con lo referido en la bibliografía. Material y métodos. Se efectuó un estudio observacional, transversal, prospectivo y controlado, entre marzo del 2002 y marzo del 2004, en 28 pacientes con diagnóstico de pénfigo vulgar que concurrieron a la Sección Ampollares de la División Dermatología y fueron evaluados posteriormente por endocrinólogos de la División Endocrinología, en el Hospital de Clínicas José de San Martín. La evaluación tiroidea fue realizada en 28 pacientes consecutivos con pénfigo vulgar y 40 voluntarios sanos (controles) apareados por sexo y edad, y consistió en palpación tiroidea, prueba de funcionalidad tiroidea mediante dosaje en sangre de las hormonas T3, T4, TSH, y prueba de autoinmunidad tiroidea mediante dosaje en sangre de anticuerpos antiperoxidasa...

Pulmonary embolism in Pemphigus Vulgaris, the need for judicious immunotherapy

Pemphigus vulgaris is a serious chronic mucocutaneous ailment. In recent decades advances in diagnostic and therapeutic measures have led to a significant decline in morbidity and mortality. However, with the advent of active and prolonged immunotherapy involving corticosteroids, there has been a rise in steroid-associated complications. This has led to significant concern globally over the sensible use of treatment regimen in pemphigus patients. We present a patient who underwent a massive pulmonary embolism following over usage of corticosteroid therapy for pemphigus vulgaris. Whilst the patient survived owing to timely assessment and aggressive surgical intervention, the need for cautious and judicious immunotherapy in pemphigus is emphasized

Endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris: immunoglobulin G heterogeneity detected by indirect immunofluorescence

Pênfigos são enfermidades auto-imunes bolhosas intraepidérmicas, onde auto-anticorpos IgG se dirigem contra glicoproteínas desmossomais. O objetivo deste estudo foi determinar o perfil de subclasses de imunoglubulina G no pênfigo foliáceo endêmico (fogo selvagem) e no pênfigo vulgar através da imunofluorescência indireta. MÉTODOS: Vinte e cinco doentes de pênfigo foliáceo endêmico (fogo selvagem), 25 de pênfigo vulgar e 25 controles sadios foram analisados através da imunofluorescência indireta, com respeito aos auto-anticorpos circulantes (imunoglobulina G total e subclasses). RESULTADOS: Nossos dados mostram uma correlação estatisticamente significativa (p<0.05) entre atividade da doença e títulos de auto-anticorpos circulantes em ambas as formas de pênfigo, ou seja, títulos negativos relacionaram-se com remissão clínica, enquanto resultados positivos correlacionaram-se com doença em atividade. A análise de subclasses de IgG mostrou que 56% dos doentes de fogo selvagem em remissão apresentaram apenas IgG4 positiva; na doença ativa, IgG4 foi a subclasse predominante, sendo positiva em 100% dos casos. Nos doentes de pênfigo vulgar, apenas 10% dos doentes em remissão apresentaram positividade exclusiva para IgG4; na doença em atividade, IgG4 esteve presente em 78-83,3% dos casos. CONCLUSÕES: A caracterização de subclasses de imunoglobulina G consiste em um instrumento de grande valia no seguimento de doentes de pênfigo, uma vez que a IgG4 é a subclasse intimamente relacionada com o reconhecimento de epítopos patogênicos, e consequentemente com atividade da enfermidade. No fogo selvagem em remissão com uma resposta homogênea 'as custas de IgG4, uma monitoração cuidadosa deve ser realizada, uma vez que isto pode significar uma maior chance de reativação.

Pénfigo herpetiforme: presentación de un caso y revisión de la literatura / Herpetiform pemphigus: case report and literature review

El pénfigo herpetiforme (PH) es una variante poco frecuente de pénfigo con rasgos clínicos, histopatológicos, inmunológicos y terapéuticos singulares. Se caracteriza por combinar figuras clínicas de dermatitis herpetiforme (DH) con inmunofluorescencia típica de pénfigo. Se expresa clínicamente como placas eritematosas, urticarianas y vesículas con distribución herpetiforme. Los hallazgos histológicos son variables, incluyendo espongiosis eosinofílica con pústulas subcórneas, asociado o no a acantolisis. Se acompaña de prurito y de buen estado general. Comparado con otros pénfigos, su curso es benigno y responde a tratamiento con dapsona, generalmente asociada a dosis bajas de corticoesteroides. Se presenta un paciente de 49 años de edad, cuyo diagnóstico definitivo de PH se realizó luego de 10 años de padecer la enfermedad (brotes extendidos en tronco y raíz de miembros de lesiones eritemato-papulo-vesiculosas, pruriginosas) y ser asistido por numerosos dermatólogos

Pénfigo vulgar bucal precedido de lesiones cutáneas: reporte de un caso / Oral pemphigus vulgaris preceded by cutaneous lesions

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 tratamento 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, UCV, 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