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Behçet syndrome in Child
See also in: Anogenital,Oral Mucosal Lesion
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Behçet syndrome in Child

See also in: Anogenital,Oral Mucosal Lesion
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Contributors: Connie Zhong, Christine S. Ahn MD, FAAD, William W. Huang MD, MPH, FAAD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Behçet disease, or Behçet syndrome, is a chronic, relapsing, inflammatory disorder of unknown etiology. It is a multisystem disease characterized by oropharyngeal and genital ulcers and ocular involvement, and there may be vascular, articular, gastrointestinal, neurologic, urogenital, pulmonary, and cardiac findings as well. Although the pathogenesis is not fully understood, it is thought to be an inflammatory reaction triggered by infectious agents such as herpes simplex 1 (HSV-1) or Streptococcus in genetically predisposed individuals. Circulating immune complexes and neutrophils are part of the pathogenesis, leading to vascular injury.

Behçet disease is relatively uncommon and is more prevalent in Japan and Middle Eastern and Mediterranean countries. While Behçet disease has an equal sex distribution overall, there is male predominance in Middle East and Mediterranean patients and female predominance in Japan and Korea. The onset of symptoms typically occurs during the third or fourth decades of life. Rarely, it can present during childhood, and usually in children with a positive family history of the syndrome. Behçet disease is strongly associated with the HLA-B51 allele, which is present in more than 80% of Asian patients with Behçet disease.

Clinically, Behçet disease is characterized by recurrent oropharyngeal and genital ulcers and ocular involvement. Oral ulcers are the initial presenting sign in up to 80% of patients and can lead to scarring, dysphagia, and odynophagia. Genital ulcers present similarly to oropharyngeal lesions; they tend to be recur less frequently but are more prone to scar formation. Potential complications include fistulae in women and epididymo-orchitis in men.

Ocular disease, seen more often in males, is characterized by episodes of bilateral, nongranulomatous anterior and/or posterior uveitis. It usually appears 2-3 years after the onset of oral and/or genital ulcers, although it can be the initial manifestation in up to 20% of cases. Approximately 1 in 4 patients with ocular disease develop blindness.

Other cutaneous manifestations include erythema nodosum-like lesions, folliculitis-like lesions, erythema multiforme-like lesions, Sweet syndrome-like lesions, subcutaneous thrombophlebitis, and palpable purpura. Nonspecific cutaneous ulcers have been observed in 3% of patients. They occur more frequently in children and tend to occur on the extremities, breasts, and interdigital and inguinal regions.

Other systemic manifestations include:
  • Musculoskeletal – arthralgia; nonerosive mono- or polyarthritis in 45%-60% of patients, affecting knees, ankles, hips, elbows, and wrists
  • Neurologic – cerebellar dysfunction, cranial nerve palsies, cerebral venous thrombosis, arterial vasculitis, aseptic meningitis
  • Vascular – deep vein thromboses, pulmonary artery aneurysms
  • Cardiac – pericarditis, myocarditis, mitral valve prolapse, intracardiac thrombosis, endomyocardial fibrosis, cardiomyopathy, coronary artery lesions
  • Gastrointestinal – mucosal inflammation and ulceration (particularly ileocecal region)
Related to Behçet syndrome is Hughes-Stovin syndrome, in which patients don't have the clinical criteria for Behçet syndrome but have a vasculitis and may have pulmonary aneurysm.

The mean age of Behçet disease onset in children is 7.8 years of age. Pediatric patients may have few symptoms, and time to diagnosis varies between 3 and 5 years. Young males tend to have worse prognosis with more neurological, ocular, and vascular involvement.

For more information, see OMIM.

Codes

ICD10CM:
M35.2 – Behçet's disease

SNOMEDCT:
310701003 – Behcet's syndrome

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Differential Diagnosis & Pitfalls

Sexual abuse – Disrupted hymen in females; may have an associated sexually transmitted disease, purpura, and bruising outside of aphthous ulcer sites. A diagnosis of aphthosis does not exclude concomitant sexual abuse, and social work should investigate when suspicion is high.

The possibility of sexually transmitted diseases should always be considered in the evaluation of genital lesions in children and, if present, sexual abuse should be suspected:
Aphthous ulcers – Oral ulcers of Behçet disease tend to recur more frequently, are larger, and can occur in multiples.

Reactive nonsexually related acute genital ulceration (RNSRAGU)

Crohn disease

Ulcerative colitis

Pyoderma gangrenosum

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Last Reviewed: 04/16/2019
Last Updated: 05/24/2019
Copyright © 2019 VisualDx®. All rights reserved.
Behçet syndrome in Child
See also in: Anogenital,Oral Mucosal Lesion
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Behçet syndrome : Abdominal pain, Painful oral ulcers, Arthralgia, Recurring
Clinical image of Behçet syndrome
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