RESUMEN
BACKGROUND: Vulvo-cervico-vaginal involvement has rarely been reported in pemphigus vulgaris (PV) and has not been reported in pemphigus foliaceus (PF). OBJECTIVES: We sought to evaluate genital lesions and Papanicolaou (Pap) smears in female patients with PV and PF. METHODS: This prospective study includes all consecutive cases of female patients with PV and PF seen from May 2009 to February 2010. Gynecologic examination was performed and Pap smears were collected for cytologic analysis from each patient. RESULTS: A total of 56 patients were given a diagnosis of pemphigus (41 PV and 15 PF). Genital involvement was observed in 9 patients with PV (22%) and the vulva was the most common genital site of involvement. Of these 9 patients, 8 presented with active skin/mucous lesions. Four of 15 patients with PF had genital lesions and vulva was the exclusive site of involvement. Three of 4 patients with PF and genital involvement also showed active cutaneous lesions. Six of 56 patients (5 PV and 1 PF) presented with atypical squamous cells of undetermined significance in Pap smear analysis. Upon further pathologic review, acantholytic cells were seen, confirming the diagnosis of pemphigus. LIMITATIONS: A small number of PF cases were studied. CONCLUSIONS: Vulvar lesions were the second most frequent site of mucous membrane PV. Herein we report the first case to our knowledge of symptomatic genital lesions in a patient with PF. Moreover, acantholytic cells in Pap smears were found in a patient with PF who was in complete remission off therapy with no clinical genital lesions and no circulating anti-desmoglein-1 and anti-desmoglein-3 autoantibodies. Gynecologic evaluation in patients with pemphigus, including a careful evaluation of Pap smears, should be recommended.
Asunto(s)
Enfermedades de los Genitales Femeninos/etiología , Pénfigo/complicaciones , Adulto , Anciano , Femenino , Enfermedades de los Genitales Femeninos/patología , Humanos , Persona de Mediana Edad , Prueba de Papanicolaou , Pénfigo/patología , Estudios Prospectivos , Enfermedades del Cuello del Útero/etiología , Enfermedades Vaginales/etiología , Frotis Vaginal , Enfermedades de la Vulva/etiología , Adulto JovenRESUMEN
O imunomapeamento, uma técnica de imunofluorescência, é o método atual mais utilizado para o diagnóstico laboratorial e a diferenciação dos principais tipos de epidermólise bolhosa hereditária, uma vez que determina o plano de clivagem na junção dermo-epidérmica das doenças mecano-bolhosas.
Immunological mapping, an immunofluorescence technique, is currently the method most used to diagnose and differentiate the principal types of hereditary epidermolysis bullosa, since this technique is capable of determining the level of cleavage of this mechanobullous disease.
Asunto(s)
Humanos , Epidermólisis Ampollosa/diagnóstico , Epidermólisis Ampollosa/clasificación , Epidermólisis Ampollosa/genética , Microscopía FluorescenteRESUMEN
Immunofluorescence is a valuable auxiliary diagnostic tool for autoimmune bullous diseases and inflammatory disorders, since their clinical and histopathologic findings may be inconclusive. It is a feasible laboratory method that requires experienced technicians and detects in situ and circulating immune deposits that may be involved in the pathogenesis of such skin diseases.
Asunto(s)
Membrana Basal/química , Técnica del Anticuerpo Fluorescente Directa , Técnica del Anticuerpo Fluorescente Indirecta , Enfermedades de la Piel/diagnóstico , Biopsia , Complemento C3/análisis , Humanos , Inmunoglobulinas/análisis , Liquen Plano/diagnóstico , Liquen Plano/patología , Lupus Eritematoso Cutáneo/diagnóstico , Lupus Eritematoso Cutáneo/patología , Pénfigo/diagnóstico , Pénfigo/patología , Porfirias/diagnóstico , Porfirias/patología , Enfermedades de la Piel/patología , Vasculitis/diagnóstico , Vasculitis/patologíaRESUMEN
A imunofluorescência é um valioso instrumento auxiliar no diagnóstico das dermatoses bolhosas autoimunes e desordens inflamatórias, uma vez que seus achados clínicos e histopatológicos podem não ser determinantes. Consiste em um método laboratorial factível, que requer profissionais técnicos experientes, e detecta imunocomplexos in situ e/ou circulantes, que podem estar envolvidos na patogênese de tais enfermidades cutâneas.
Immunofluorescence is a valuable auxiliary diagnostic tool for autoimmune bullous diseases and inflammatory disorders, since their clinical and histopathologic findings may be inconclusive. It is a feasible laboratory method that requires experienced technicians and detects in situ and circulating immune deposits that may be involved in the pathogenesis of such skin diseases.
Asunto(s)
Humanos , Membrana Basal/química , Técnica del Anticuerpo Fluorescente Directa , Técnica del Anticuerpo Fluorescente Indirecta , Enfermedades de la Piel/diagnóstico , Biopsia , /análisis , Inmunoglobulinas/análisis , Liquen Plano/diagnóstico , Liquen Plano/patología , Lupus Eritematoso Cutáneo/diagnóstico , Lupus Eritematoso Cutáneo/patología , Pénfigo/diagnóstico , Pénfigo/patología , Porfirias/diagnóstico , Porfirias/patología , Enfermedades de la Piel/patología , Vasculitis/diagnóstico , Vasculitis/patologíaRESUMEN
Immunological mapping, an immunofluorescence technique, is currently the method most used to diagnose and differentiate the principal types of hereditary epidermolysis bullosa, since this technique is capable of determining the level of cleavage of this mechanobullous disease.
Asunto(s)
Epidermólisis Ampollosa/diagnóstico , Epidermólisis Ampollosa/clasificación , Epidermólisis Ampollosa/genética , Humanos , Microscopía FluorescenteRESUMEN
UNLABELLED: Pemphigus are autoimmune intraepidermal blistering diseases in which immunoglobulin G (IgG) autoantibodies are directed against desmosomal glycoproteins. The aim of this study was to determine the IgG subclass profile of endemic pemphigus foliaceus (fogo selvagem) and pemphigus vulgaris utilizing indirect immunofluorescence. PATIENTS AND METHODS: Twenty-five patients with pemphigus vulgaris, 25 with endemic pemphigus foliaceus (fogo selvagem), and 25 healthy controls were analyzed by indirect immunofluorescence for circulating autoantibodies (total IgG and its subclasses). RESULTS: Our data revealed a significant correlation (P <.05) of disease activity and autoantibody levels in both forms of pemphigus, i.e., negative titers related to clinical remission, whereas positive results related to active disease. Immunoglobulin G subclass analysis in fogo selvagem demonstrated that in patients in remission, 56% showed positive immunoglobulin G4; in active disease, immunoglobulin G4 was the predominant subclass (100% positive in all cases). The IgG subclass profile in pemphigus vulgaris showed that in patients in remission, only 10% were positive for immunoglobulin G4; in active disease, positivity for immunoglobulin G4 was present in 78% to 88% of the cases. CONCLUSION: Subclass characterization of immunoglobulin G autoantibodies is a useful tool for pemphigus follow-up, since immunoglobulin G4 (IgG4) is the subclass that is closely related to recognition of pathogenic epitopes, and consequently with disease activity. Careful monitoring should be performed for fogo selvagem in clinical remission with a homogeneous IgG4 response, since this may indicate more frequent relapses.
Asunto(s)
Autoanticuerpos/sangre , Inmunoglobulina G/sangre , Pénfigo/inmunología , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Casos y Controles , Desmogleínas/inmunología , Femenino , Técnica del Anticuerpo Fluorescente Indirecta , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana EdadRESUMEN
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.