Endemic pemphigus in the peruvian Amazon: epidemiology and risk factors for the development of complications during treatment / Pênfigo endêmico na Amazônia peruana: epidemiologia e fatores de risco para o desenvolvimento de complicações durante o tratamento

BACKGROUND: Pemphigus is an autoimmune blistering disease. According to a report, in areas of endemic pemphigus foliaceus (EPF) in Peru there are cases of pemphigus vulgaris with epidemiologic, clinical and histopathologic characteristics similar to those of "endemic pemphigus vulgaris" (EPV) in Brazil. OBJECTIVES: To determine the clinical and epidemiologic characteristics of endemic pemphigus and the risk factors of patients for developing complications during treatment. METHODS: A study was carried out from July 2003 to March 2008. The study population was 60 patients with EPF and 7 patients with EPV evaluated in hospitals and clinics in the Peruvian Amazon and Lima. A multivariate analysis was carried out using binary logistic regression. RESULTS: The average age of EPF patients was 31.4 years; 55% were men; 60% presented the generalized clinical variant. Non-compliance with the treatment was seen in 57.1% of the patients. Thirty-five percent presented complications (e.g. pyodermitis and pyelonephritis) during treatment. The risk factors for developing complications during treatment were non-compliance with the treatment and having the generalized clinical form. In the EPV group, the average age was 21.7 years; 71.4% were men. All patients presented with the mucocutaneous clinical variant and the initial presentation consisted of oral mucosa lesions; 71.4% presented complications during treatment, pyodermitis being the most frequent. CONCLUSIONS: Non-compliance with the treatment and the generalized clinical form are risk factors for the development of complications during treatment of patients with EPF. Peru indeed has EPV cases with epidemiologic characteristics similar to EPF. Living in a rural area may represent a risk factor for the development of complications during treatment of patients with EPV.

FUNDAMENTOS: O pênfigo é uma doença auto-imune bolhosa. Segundo um relatório, em áreas de pênfigo foliáceo endêmico no Peru há casos de pênfigo vulgar com características epidemiológicas, clínicas e histopatológicas semelhantes às do "pênfigo vulgar endêmico" no Brasil. OBJETIVOS: Determinar as características clínicas e epidemiológicas do pênfigo endêmico e os fatores de risco para o desenvolvimento de complicações durante o tratamento. MÉTODOS: Um estudo foi realizado de julho de 2003 a março de 2008. 60 doentes de pênfigo foliáceo endêmico e 7 de pênfigo vulgar endêmico foram avaliados em hospitais e clínicas na Amazônia peruana e em Lima. Uma análise multivariante foi feita usando regressão logística binária. RESULTADOS: A idade média dos doentes de pênfigo foliáceo endêmico foi 31,4 anos; 55% eram homens, 60% apresentavam a forma clínica generalizada. 57,1% nao cumpriram o tratamento. 35% apresentaram complicações (por exemplo, piodermites e pielonefrite). Os fatores de risco foram não cumprir o tratamento e ter a forma clínica generalizada. No grupo pênfigo vulgar endêmico, a idade média foi 21,7 anos; 71,4% eram homens. Todos os pacientes apresentavam a variante clínica mucocutânea e a apresentação inicial consistia de lesões da mucosa bucal; 71,4% apresentaram complicações durante o tratamento, piodermites sendo a mais freqüente. CONCLUSÕES: Não cumprir o tratamento e ter a forma clínica generalizada são fatores de risco para o desenvolvimento de complicações durante o tratamento de pênfigo foliáceo endêmico. Peru realmente tem casos de pênfigo vulgar endêmico com características epidemiológicas semelhantes às do pênfigo foliáceo endêmico. Viver numa área rural pode ser um fator de risco para o desenvolvimento de complicações.

Enfermedad de Still en el Hospital Nacional Dos de Mayo / Still's disease and adult still's disease at Dos de Mayo National Hospital

Objetivo: Determinar las características clínicas, epidemiológicas e histopatológicas de la Enfermedad de Still en el Hospital Nacional Dos de Mayo. Pacientes y Métodos: Estudio descriptivo, retrospectivo y transversal que incluyó pacientes con Enfermedad de Still atendidos en el Servicio Académico Asistencial de Dermatología de la UNMSM (Sede Hospital Nacional Dos de Mayo) desde marzo 1998 hasta julio del 2006 diagnosticados de acuerdo a los criterios de Yamaguchi. Resultados: Se presentaron 7 casos de Enfermedad de Still, tres de los cuales correspondieron a enfermedad de Still del adulto. Seis pacientes fueron mujeres y uno varón, la edad promedio fue de 24,7 +/- 15,2 años (12 a 53 años) y el tiempo de enfermedad promedio de 3,8 meses. Se encontraron lesiones maculopapulares eritematosas, cuya ubicación fue: abdomen, espalda y miembros inferiores (5 pacientes), miembros superiores (4 pacientes), tórax (3 pacientes) y el rostro (2 pacientes). Las lesiones fueron pruriginosas en 3 pacientes. El estudio histopatológico de piel demostró dermatitis perivascular superficial. Conclusiones: La Enfermedad de Still es una entidad clínica de presentación rara y con notorio compromiso sistémico y cutáneo.

Objective: To determine the histopathology, epidemiological and clinical characteristics of the Still's disease at Dos de Mayo National Hospital (HNDM). Patients and methods: Descriptive, retrospective and transversal study. Patients with Still's disease were seen in the Dermatology teaching division of the San Marcos University (HNDM) from March 1998 to July 2006. Results: Seven patients with Still's disease were diagnosed according to Yamaguchi criteria, 3 of them were adult-onset Still's disease. Six patients were female and one male. The average age was 24,7 ± 15,2 years (12 to 53 years) and the average of disease duration average was 3,8 months. We found maculopapular and erythematosus lesions on the inferior abdomen, back and lower extremities (5 patients), upper extremities (4 patients), thorax (3 patients) and on the face (2 patients). The lesions were itchy in 3 patients. The histopathologic study of skin showed superficial perivascular dermatitis. Conclusions: Still´s disease is a clinical entity of rare presentation and with well-known systemic and cutaneous involvement.