Oral pemphigus treated with minipulse therapy

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.

Epidemiology of pemphigus in Tehran, Iran: a 20-year retrospective study

Pemphigus is a chronic autoimmune and vesiculobollous disease that can affect skin and different mucous membrane surfaces. Primary manifestations occur in oral cavity in almost 60% of cases. The purpose of the present study was to evaluate the epidemiology of pemphigus in Tehran, Iran in a 20-year period. A retrospective study was conducted on the records of 1560 patients diagnosed with different types of pemphigus in Razi Hospital of Dermatology in Tehran from March 1985 to March 2005. A questionnaire was prepared to collect information regarding age, sex, bedridden duration, pemphigus subtype, sites of involvement, recurrence and mortality rate. Data was analyzed using chi-square test with significant level of P < 0.05. There was a female predominance with a male to female ratio of 1:1.53. In nearly half of the patients, only the oral mucous membranes were affected. One hundred and fifty had only skin lesions and 261 cases had both skin and oral mucosal lesions. Involvement of esophageal and vaginal mucous membranes without skin lesions was observed in 150 patients and 298 cases had esophageal and vaginal mucosal involvement as well as skin lesions. Pemphigus vulgaris was the most common type, with the mean age of 44.6 years. Oral mucous membrane was the most frequent location where pemphigus vulgaris was observed. 1265 patients recovered which 52.2% of them had only oral lesions. Average of bedridden duration was 2.9 months. The highest recurrence rate was seen in patients with skin lesions exclusively. There was a significant difference between recurrences of lesions and location of involvement [P < 0.05]. Thirty six patients had died from of the disease. The mean age of the disease onset in the present study was found to be a decade earlier than the other parts of the world. Recurrence and mortality rates were lower in patients with only oral lesions and their prognosis was better

Treatment of pemphigus vulgaris and pemphigus foliaceus: experience with 71 patients over a 20 year period

Forty one cases of pemphigus vulgaris and thirty cases of pemphigus foliaceus were investigated at Hospital Universitário Clementino Fraga Filho from 1978 to 1999. They were divided into two treatment groups: one group received up to 100 mg of oral prednisone daily and the other group received >120 mg daily. The dose up to 100 mg provided good initial control of pemphigus vulgaris and pemphigus foliaceus and did not increase the mortality rate associated to disease. The dose >120 mg induced higher morbidity. These data allowed us to establish a regimen of oral prednisone (1-2 mg/kg/daily) with maximum of 120 mg daily in the treatment of pemphigus vulgaris and pemphigus foliaceus

Estudio retrospectivo de pacientes con pénfigo, en un período de diez años / Retrospective study of patients with pemphigus, during an 10 years period

Se presentan 92 pacientes con pénfigo internados durante un período de diez años. De ellos, 50 eran masculimnos y 42 femeninos. Observando como formas clínicas: pénfigo vulgar (79 pacientes), pénfigo vegetante (2 pacientes), pénfigo foliáceo no endémico (6 pacientes) y pénfigo eritematodes (5 pacientes). El 60,8 por ciento correspondieron a formas graves, el 20,6 por ciento a formas moderadas y 18,4 por ciento a formas leves. Más del 50 por ciento de los pacientes graves requirieron dosis superiores a 180 mg/día de 16 ß metilprednisona. La terapéutica esteroidea fue asociada con inmunosupresores (ciclofosmida, azatioprina y metotrexato), daps u oro, mientras que en 10 pacientes no se agregó ninguna medicación y en otros 10 se asoció más de un fármaco en forma sucesiva. Diez pacientes fallecieron, el 50 por ciento por sepsis. Del análisis de la casuística surgen algunas conclusiones: la mejor respuesta terapéutica en pacientes vírgenes de tratamiento; la utilidad del empleo de dosis altas de esteroide desde el inicio; el comportamiento biológico agresivo de algunos pénfigos superficiales; el valor predictivo de la edad y enfermedades concomitantes preexistentes con respecto a la aparición de complicacionmes; el desarrollo de sepsis como primera causa de muerte; la similar incidencia de complicaciones infecciosas, metabólicas y hematológicas, y la trascendencia de enfermedades cardiovasculares preexistentes como factor condicionante del óbito

Pemphigus: epidemiological survival characteristics of 72 patients

From 1st October, 1991 to 30th September, 1992 an observational study was conducted on pemphigus patients presenting to the department of dermatology, Mayo Hospital, Lahore in order to document the epidemiological and survival characteristics. A total of 72 pemphigus patients were recorded. Thirty eight of these were already known to have the disease while 34 were seen for the first time. A majority of patients[76%] were from lower socioeconomic class. Pemphigus vulgaris was the most common type, accounting for 83% of the total patients, with female to male ratio of 4.4:1. Most of the patients were in third and fourth decades of life. Eight patients[11%] complained of exacerbation during summer and three [4%] had history of photosensitivity. In two patients disease relapsed at the site of trauma. Eleven deaths [15% mortality] occurred during this period either due to the disease itself or disease related complications