Behçet syndrome

doi:10.1016/S0022-3476(85)80611-9

The Journal of Pediatrics

Volume 107, Issue 1, July 1985, Pages 41-43

https://doi.org/10.1016/S0022-3476(85)80611-9 Get rights and content

Behçet syndrome, a multisystem disorder characterized by ocular, mucocutaneous, articular, vascular, gastrointestinal, and neurologic abnormalities, is described in six pediatric patients. The patients ranged in age from 2 months to 11 years at time of onset. Several years were usually required before additional manifestations of the disease occurred. Aphthous ulceration was present in all six patients, arthritis in three, erythema nodosum in four, sterile cellulitis in three, gastrointestinal manifestations in five, neurologic manifestations in two, and genital or perianal ulcerations in three; ocular involvement was present in only one. There were no diagnostic laboratory studies, and, as in the adult population, no cause for Behçet syndrome was found. Patients had a variable response to corticosteroid therapy. Two patients who had significant morbidity and who responded poorly to corticosteroid therapy also received chlorambucil therapy, which appeared to provide improved control of signs and symptoms. Based on the prolonged interval between onset and the appearance of complete manifestations. Behçet syndrome may be more common in children than previously reported.

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Cited by (51)

Uveitis of children
2012, Journal de Pediatrie et de Puericulture
Étayer les particularités épidémiologiques et cliniques de l’uvéite de l’enfant et analyser leur profil évolutif.
Il s’agit d’une étude rétrospective incluant 32 enfants (43 yeux), présentant une uvéite, colligés sur une période de 11 ans allant de janvier 1998 à décembre 2010 au service d’ophtalmologie, CHU La Rabta de Tunis, Tunisie. Tous les patients ont bénéficié d’un examen ophtalmologique complet, d’une angiographie rétinienne en fluorescence et d’une tomographie en cohérence optique en l’absence de troubles des milieux. Une enquête étiologique a été réalisée dans tous les cas.
L’âge moyen de nos patients était de 11,67 ans (sept à 17 ans) avec une prédominance masculine (sex-ratio M/F de 2,5). Le suivi moyen était de 22,53 mois. L’uvéite était bilatérale dans 34,4 % des cas. Il s’agissait d’une uvéite antérieure isolée dans 25,6 % des cas, d’une uvéite postérieure isolée dans 39,5 % des cas et d’une panuvéite dans 34,9 % des cas. Les uvéites infectieuses représentent l’étiologie la plus fréquente (65,6 % des cas) dominée par la rétinochoroïdite toxoplasmique (34,4 %). L’arthrite chronique juvénile constituait la principale étiologie des uvéites antérieures isolées (54,5 % des cas). La maladie de Behçet (3,12 %) et le syndrome de Voght Koyanagy Harada (6,25 %) ont été rarement observés. Aucune étiologie n’a été identifiée dans 15,62 % des cas. Des complications oculaires ont été observées dans 60,5 % des cas dominées par les synéchies iridocristalliniennes (18,6 %) et la cataracte (14 %). Une cécité légale a été notée dans 6,9 % des cas.
L’uvéite de l’enfant est une pathologie rare, mais potentiellement cécitante. Les causes infectieuses sont fréquentes et doivent être éliminées en premier lieu. Une prise en charge précoce et multidisciplinaire reste le seul garant pour préserver le pronostic visuel.
To analyze the pattern of childhood uveitis.
The study included 32 patients with uveitis examined from January 1998 to December 2010. Complete ophthalmic examination, fluorescein angiography and optic coherence tomography were performed in all cases. Standard diagnostic criteria were employed for all syndromes or entities of uveitis.
The mean age at onset of uveitis was 11.67 years. The male-to-female ratio was 2.5. The uveitis was bilateral in 34.8% of patients. Mean follow-up was 22.53 months. Anterior uveitis represented 25.6% of cases, posterior uveitis 39.5%, and panuveitis 34.88%. Infectious uveitis (65.6%) was the most frequent type of inflammation and toxoplasmosis (34.4%) being the most frequent cause. Idiopathic uveitis was found in 15.6% of patients. Juvenile idiopathic arthritis was found in 9.37% of cases. Ocular complications occurred in 60.5% of affected eyes. Posterior synechiae (18.6%) and cataract (14%) were the most common complications. Legal blindness was observed in 6.9% of the affected eyes.
Uveitis in childhood is a rare but severe condition. Infectious uveitis was the leading cause of uveitis in our study. Prompt diagnosis and treatment is mandatory to minimize the risk of blindness.
Concurrence of Incontinentia Pigmenti and Behçet's Disease
2010, Journal of the Chinese Medical Association
We report here a rare case of incontinentia pigmenti (IP) in a 10-year-old girl who developed Behçet's disease. IP was diagnosed in infancy and Behçet's disease was diagnosed at 10 years of age. The initial presentations of Behçet's disease were spiking fever and recurrent painful oral and genital ulcers that were refractory to antibiotics. After corticosteroid treatment, her fever subsided and ulcers subsequently healed. The patient's mother and sister were also diagnosed with IP. Her mother had suffered from Behçet's disease since her teenage years and it was complicated with colon perforation. Although there are several reports on the combination of IP and Behçet's disease, this is the first reported case of a family with such concurrence.
Behçet's disease in child: 3 paediatric cases
2006, Journal de Pediatrie et de Puericulture
La maladie de Behçet (MB) de l'enfant est aujourd'hui mieux décrite grâce à une augmentation constante de la littérature sur le sujet. L'objectif de ce travail est de faire le point sur la maladie dans ses aspects pédiatriques et surtout sur l'intérêt d'un traitement initial, précoce et rapidement agressif. Les auteurs rapportent trois observations pédiatriques avec des manifestations diverses et variées. Deux enfants parmi les trois, âgés respectivement de sept et huit ans, ont présenté une atteinte oculaire sévère associée à des lésions cutanéomuqueuses très évocatrices de la maladie avec une évolution heureuse. Cela est dû à un diagnostic précoce et à un traitement initial agressif. La présentation clinique chez le troisième enfant âgé de 15 ans a été uniquement cutanéomuqueuse. La MB est caractérisée par un très grand polymorphisme clinique. Le pronostic demeure sombre et conditionné par l'atteinte oculaire particulièrement sévère dans cette tranche d'âge. Un traitement précoce et agressif peut limiter les séquelles particulièrement importantes dans cette tranche d'âge.
Nowadays, Behcet's disease in child is better described since increasing number of papers are clearly reported the pathology. The goal of this paper is to report the pediatric aspects, especially the importance of fast aggressive, precocious and initial treatment. We report three pediatric cases with various manifestations. Two children among three are 7 and 8 years old respectively; both have severe ocular affections associated to cutaneomucosal lesions evoking the pathology with favorable evolvement; this was the result of initial aggressive and precocious treatment. The third patient was 15 years old. He was only suffering cutaneomucosal lesions. The Behcet's disease is characterized by its huge clinical polymorphism. The prognosis is still poor and depends on the ocular affection which remains severe in this age range. The initial aggressive and precocious treatment might limit the sequels which are major, especially in the reported age range.
Behçet's disease
2005, Textbook of Pediatric Rheumatology
Fatal hemoptysis from Behcet's disease in a child
2002, Cardiovascular Pathology
Objective: To present a case of pulmonary artery aneurysms (PAAs) in a child with Behcet's disease (BD). Nature of the study: Case report and review of the literature. Materials and methods: The patient had a complete autopsy, drug screen and serological testing. A medical history was obtained and the literature was reviewed. Results: A 10-year-old African–American boy had massive hemoptysis while at home and died shortly thereafter in the emergency department of a local hospital. He had had two other episodes of hemoptysis in the past 2 months. At autopsy, he had bilateral inflammatory aneurysms of the lower lobe branches of the pulmonary arteries. The left aneurysm had eroded into the adjacent bronchus and accounted for the fatal hemoptysis. The right ventricle had endocardial fibrosis. Systemic vasculitis was absent. The child did not have oral, genital, eye or skin lesions at autopsy, or by clinical history. Based on the autopsy findings, a diagnosis of BD was made. Conclusions: We present the very unusual case of a 10-year-old African–American boy who died from hemoptysis resulting from bilateral pulmonary aneurysms caused by BD.
Oral disorders: Unapproved treatments or indications
2000, Clinics in Dermatology

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^*: Supported by the Arthritis Foundation and by the Division of Research Resources, National I(nstitutes of Health.

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Original articleBehçet syndrome*

Am J Med

Semin Arthritis Rheum

Behçet's syndrome. Clinical and immunologic features

Clinical studies on Behçet's syndrome

Am Rheum Dis

Behçet's disease: Report of 10 cases, 3 with new manifestations

Ann Intern Med

Recurrent oral ulcers and Behçet's syndrome: Pathological, immunological and clinical studies

Behçet's disease presenting as chronic aphthous stomatitis in a child

Pediatrics

Behçet's syndrome with arthritis

Ann Rheum Dis

Original article
Behçet syndrome*