Behçet Syndrome

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WHO-ICD-10 version:2010

Diseases of the musculoskeletal system and connective tissue

Systemic connective tissue disorders

OMIM Number

109650

Mode of Inheritance

Familial cases reported, but probably not Mendelian

Description

Behcet disease is a chronic inflammatory multicystic vasculitic syndrome, characterized by painful canker sores and ulcers in the mouth, genitals and/or eyes. BD is considered, at least in part, a genetic disease, due to the small number of familial cases of the syndrome seen around the world. This genetic susceptibility has been shown to be associated with certain genes of the major histocompatibility complex (MHC). The most prominent of these are: the HLAB5, HLAB51, TNF, and MICA genes that are linked to the ocular symptoms. Similarly, HLAB12 and HLAB27 have been shown to be associated with the mucocutaneous and joint lesions, respectively. 

 

Epidemiology in the Arab World

View Map
Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
109650.G.4JordanUnknown Anterior uveitis, ; Posterior uveitis... NM_000201.2:c.1405A>GVerity et al. 2000 Association study wi...
109650.G.1LebanonUnknown Anterior uveitis, ; Posterior uveitis... NM_000201.2:c.1405A>GHeterozygousChmaisse et al. 2006 Group of 17 patients
109650.G.2LebanonUnknown Anterior uveitis, ; Posterior uveitis... NM_000201.2:c.1405A>GHomozygousChmaisse et al. 2006 Group of 6 patients
109650.G.5Saudi ArabiaUnknown Anterior uveitis, Posterior uveitis... NM_005514.7:c.97T>CAndo et al. 1997 Family with unknown ...

Other Reports

Algeria

Guenane et al, 2006, studied 24 patients with Behcet's disease to investigate the role IFN-gamma, IL-12, IL-10 and nitric oxide in the immunopathologic mechanisms of uveitis. 

Bahrain

Ebrahim et al, 2001, found nine Bahraini cases of Behcet's disease over a 15 year period, giving a disease prevalence of 2.23 per 100,000 individuals.

[Ebrahim RA, Alawi RA, Farid E. Behcet's disease in Bahrain, clinical and HLA findings. Bahrain Med Bull. 2001; 23(1):27-9.]

Eritrea

Chaloupka et al, 2003, reported the case of a 13-year-old Eritrean boy with recurrent venous thrombosis of the dural sinus who was diagnosed with Behcet's disease.  

Kuwait

Mousa et al, 1986, studied 29 cases of Behcet's disease and calculated its prevalence in the Kuwaiti population to be 1.35:100,000 individuals. It was also suggested that Kuwaiti Bedouins were resistant to the disease.

Ozoebe, 1996, carried out a case-control study and found that thrombomodulin, thrombin-antithrombin III complex and plasminogen activator inhibitor levels were significantly elevated in Behcet's patients compared to controls.

[Ozoebe OI. Soluble thrombomodulin and fibrinolysis in Behcet's disease. Med Princ Pract. 1996; 5(3): 146-50.]

Farah et al, 2006, studied 37 Arab Behcet cases and suggested that oral and genital ulcers should be looked for in patients presenting with benign intracranial hypertension.

Lebanon

Hamdan et al, 2006, carried out a retrospective review of 90 patients with Behcet's disease. 

Ziade and Awada, 2006, described two cases of late-onset Behcet's disease in Lebanon. The first, a 70 year old Lebanese male, suffered from oligoarthritis, uveitis, oral ulcerations, ear chondritis, pseudofolliculitis and pustular rash, while a 72 year old Lebanese female exhibited posterior uveitis, retinal vasculitis and bipolar cutaneous ulcerations. 

Arayssi et al (2009) found that KIR3DP1*001/002 prevalence was significantly different between Lebanese patients with Behcet's disease as compared to controls. 

Oman

Banodkar and Al-Suwaid (1997) studied 25 Behcet patients and found that the common manifestations were oro-genital ulcers, eye and CNS involvement while the most common skin manifestation was follicular pustular eruptions.

[Banodkar DD, Al-Suwaid AF. Behcet's disease in Oman. Oman Med J. 1997; 13(4):6-13.]

El-Ageb et al, 2002, described the clinical profile of 34 patients affected with Behcet's disease. 

Gowri and Nair, 2006, found Behcet's disease to be a rare cause of vulval ulcer in a perimenopausal woman.

Saudi Arabia

Al Izzi et al, 2010, described a 33-year-old Arab male with Behcet syndrome and severe idiopathic dilated cardiomyopathy (DCM) associated with complete atrioventricular block. 

Yabuki et al, 1999, genotyped 13 Saudi Behcet patients for HLA-A and HLA-B alleles. It was found that the B51 antigen was present in a significantly higher frequency among patients than controls. 

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