Behçet Disease – Clinical manifestations of 12 patients of an Autoimmune Disease Unit
Cláudia Costa, Rui Castanheira, Fátima Coelho, Carlos Dias
Resumo
Os autores apresentam um estudo retrospectivo de 12 doentes com doença de Behçet (DB). Todos os doentes cumpriam os critérios de diagnóstico de DB (International Study Group for BD, 1990). Todos os doentes eram Portugueses e caucasianos. Dos 12 doentes, 9 eram do sexo feminino e 3 do sexo masculino. A idade média de início da DB foi 31.3 anos. Todos os doentes apresentavam úlceras orais recorrentes e 66.7%, também, úlceras genitais. Nove doentes (75%) apresentavam envolvimento ocular. As manifestações dermatológicas surgiram em 58.3%. Envolvimento neurológico em 16.7%. As manifestações osteoarticulares estiveram presentes em 75%. Foi determinado o HLA em todos os doentes; o HLA-B51 foi positivo em 2 doentes do sexo feminino. Em 7 doentes havia história familiar de úlceras orais recorrentes mas apenas 1 com DB. A evolução clínica esteve marcada pelo desenvolvimento de amaurose num olho de uma doente com panuveíte.
Palavras-chave:
Doença de Behçet, uveítes, úlceras orais, úlceras genitais, eritema nodoso, vasculite, aftas.
Abstract
The authors present a retrospective study of 12 patients with Behçet’s disease. All patients fulfilled diagnostic criteria for BD (International Study Group for BD, 1990).
All patients were Portuguese and Caucasians. There were 12 patients, 9 female and three male. Mean age at onset of BD was 31.3 years. All patients presented recurrent oral ulceration and 66.7% also had genital ulceration. Nine patients (75%) had ocular involvement. Dermatological manifestation was seen in 58.3%. Neurological involvement appeared in 16.7%. Osteoarticular manifestations were presents in 75%. HLA class I alleles were determined in all patients; HLA-B51 was present in two female patients. Seven patients had familiar history of recurrent oral ulceration but only 1 with BD. Evolution was marked by one eye blindness in one female patient with panuveitis.
Keywords:
Behçet disease, uveitis, oral ulceration, genital ulceration, erythema nodosum, vasculitis.