Doença de Behçet na infância

• Autor(a) principal: Albuquerque, Patrícia R. de [UNIFESP]
• Data de Publicação: 2002
• Outros Autores: Terreri, Maria Teresa Ramos Ascensão [UNIFESP], Len, Claudio Arnaldo [UNIFESP], Hilário, Maria Odete Esteves [UNIFESP]
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Institucional da UNIFESP
• Texto Completo: http://dx.doi.org/10.1590/S0021-75572002000200011; http://repositorio.unifesp.br/handle/11600/1393
• topic: Behcet s disease; recurrent ulcers; doença de Behçet; aftas recorrentes

Objective: to make Pediatricians aware of the fact that they must investigate Behcet's disease while performing differential diagnosis of recurrent aphthous stomatitis, even though it is a vasculitis of rare occurrence in early life. Methods: between June 1996 and December 2000, we retrospectively evaluated seven patients of our Pediatric Rheumatology Unit. Demographic, clinical, and laboratory data as well as data regarding treatment and follow-up were presented.Results: five out of seven patients were female (71.4%), four were non-Caucasian (57.1%), the mean age at onset was 8 years and 11 months (variation of 6 months to 13 years and 8 months), the mean period until diagnosis was 2 years and 3 months (variation of 2 months to 8 years) and the mean follow-up period was 4 years and 2 months (three patients without follow-up). The major criteria of diagnosis were: oral ulcers in seven patients (100%), genital ulcers in three patients (42.8%), ophthalmic alterations in four patients (57.1%) cutaneous vasculitis in one patient (14.2%) and positive pathergy test in one patient (14.2%). The minor criteria were: arthralgia/arthritis in five patients (71.4%), family history in two patients (28.5%), and sagittal sinus thrombosis in one patient (14.2%). The initial symptoms included recurrent aphthous stomatitis (more than three painful aphthous ulcers episodes in the period of 1 year), genital ulcers, arthralgia, fever and weight loss. The laboratory findings were: mild anemia in 1/6 patients, ESR>25 in 3/6 patients, increased serum gammaglobulin level in 2/4 patients, B5 histocompatibility antigen in 2/7 patients. The treatment included corticosteroids for 5/7 patients (4 oral, 1 intravenous and one local use), thalidomide for 4/7 patients, colchicine for 2/7 patients and dapsone for 1/7 patient. The outcome was favorable in 4/6, and 3/6 patients presented relapse.Conclusion: our results confirm the importance of considering the diagnosis of Behcet's disease in patients with recurrent oral and genital ulcers.