Evaluación inmunopatológica del pénfigo foliáceo endémico luego de seis meses de tratamiento: seguimiento de cinco casos de la Amazonía peruana

Objetivo. Describir la respuesta inmunopatologica de cinco pacientes peruanos con penfigo foliaceo endemico antes y seis meses despues de la terapia con corticosteroides sistemicos. Material y metodos. Estudio descriptivo de tipo serie de casos. Los pacientes fueron sometidos a examen fisico, se obtuvodatos cinicos y, luego, una muestra de sangre, a la que se le realizo inmunofluorescencia indirecta (IFI). Se uso como sustratos esofago de mono y piel humana normal, Elisa IgM antidesmogleina 1, Elisa IgG antidesmogleina 1 (total y subclases), inmunoprecipitacion (IP) antidesmogleina 1 y antidesmogleina 3. Los pacientes recibieron tratamiento con corticosteroides sistemicos por seis meses obteniendose nuevamente una muestra de sangre para estudiar su evolucion inmunopatologica. Resultados. Al examen fisico inicial tres pacientes presentaban la forma clinica generalizada y dos, la localizada; a los seis meses de tratamiento solo un paciente presentaba lesiones activas debido a que su tratamiento habia sido irregular. La IgManti-DSG-1 no mostro variaciones importantes manteniendose positiva en un paciente y haciendose positiva en un paciente inicialmente negativo. El Elisa para anticuerpos IgG4 se redujo notablemente en todos los pacientes incluidos los que tenian tratamiento irregular. De los dos pacientes que presentaban anticuerpos anti-DSG-3 (Elisa) uno redujo sus valores indices y elotro se hizo negativo; mientras que un paciente inicialmente negativo desarrollo al final del tratamiento anti-DSG-3. Conclusion. La respuesta antidesmogleina 1 IgG total e IgG4 asi como la respuesta antidesmogleina 3 muestran reduccion de sus valores indices con tendencia a la negativizacion luego de seis meses de tratamiento lo cual es evidenciado tambien con la IFI. La respuesta IgM antidesmogleina 1 no mostro 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.