Clinical Analysis of 40 Cases of childhood‐Onset Behçet's Disease

Abstract

Behçet's disease is a chronic, relapsing, idiopathic, multi-system condition characterized by mucocutaneous, ocular, gastrointestinal, articular, vascular, urogenital, and neurologic involvement. Usually the onset occurs between 20 and 30 years of age; it is very uncommon in children. We analyzed 40 cases of childhood-onset Behçet's disease. The male:female ratio was 0.67. The most frequent major sign was oral ulceration, appearing in all patients. Other major signs were genital ulcers (33 patients), skin lesions (29), and ocular lesions (11). The average time interval between the initial oral ulceration and the second major manifestation was 8.8 years. After the second major manifestation, the third and fourth features rapidly developed within one to two years. The most frequent minor sign was arthritis, occurring in 11 of 40 patients. Intestinal, neurologic, and renal involvement was also present. From our results, we concluded that oral ulceration, which is the most common initial manifestation, should not be neglected in children, since it may signal Behçet's disease.

Survey and Validation of the Criteria for Behcet's Disease Recently Used in Korea: a Suggestion for Modification of the International Study Group Criteria

“…Twelve comprised the other 3 types except for the possible type and the remaining 4 comprised only two types, the complete and incomplete types. The ISG criteria were first employed in 1994 in Korea (16), and although this criteria has been increasingly used year after year, the Japanese criteria has still been popularly adopted (Fig. 1).…”

Uveitis in juvenile Behçet's disease: clinical course and visual outcome compared with adult patients

“…One of the consistent findings in the literature was a long interval (up to 9 years) between initial appearance of the clinical signs and diagnosis. 1,13,19 Thus, it is crucial to identify the clinical course, make the diagnosis, and initiate aggressive treatment even before the disease is fully expressed. The retrospective nature of this study and the small number of cases precluded conclusions regarding the preferred choice of drugs.…”

Doença de Neuro-Behçet de início na infância

“…Na população adulta, segundo a literatura, há predominância do sexo masculino sobre o feminino (12) , ocorrendo mais freqüentemente entre 18 e 40 anos de idade. Na infância, a doença não é freqüente e é caracterizada por uma baixa incidência de lesão ocular e neurológica e alta incidência de envolvimento intestinal (13,14) . Esse paciente não apresentou comprometimento cutâneo e intestinal, sendo os componentes marcantes de sua doença, o ocular e o neurológico.…”

“…Os agentes citotóxicos (azatioprina, ciclofosfamida, metotrexato, clorambucil), associados aos corticosteróides, são utilizados nos casos de maior gravidade, como nos casos de enterite de delgado grave e vasculite do SNC (9,13,14) . Sendo assim, chamamos a atenção para a possibilidade de envolvimento neurológico na doença de Behçet juvenil, uma vez que representa importante causa de mortalidade, invalidez e seqüelas neurológicas.…”