Understanding Behçet Syndrome: Causes & Symptoms

Behçet syndrome or disease is a disease of unknown causes or origin, though it has been around for some time. Behcet syndrome is characterised by ulcers in the mouth, genitals and eyes, and in the vascular system.¹

Behçet syndrome is thought to be a multi-system inflammatory disease with a variety of symptoms and manifestations. This condition can be complex to diagnose, since it based only on a set of clinical criteria, and these vary by geography.²

Behçet syndrome is thought to occur in between 0.33 and 370 cases per 100,000 people, but there are places where the incidence is much higher than others.

Who gets Behçet disease?

Behçet disease is more common in Turkish, Asian and Middle Eastern people.^3,4 In Asia, it is more common in females, but in the Middle East, more males develop this disease. Behçet disease appears to be more severe in males.

Olmsted County, Minnesota – 5 per 100,000 people
Turkey – 80-370 per 100,000 people
Japan, Korea, China, Middle East – 13-20 per 100,000 people
Northern Spain – 0.66 per 100,000 people
Germany – 2.26 per 100,000 people

Age of onset of Behçet disease

Typically Behcet syndrome appears in the late third and early fourth decades of life and during childhood, with the Turkish average age of onset being 11.7 years, with the disease rarely occurring in kindergarten-aged children.⁵

Signs, symptoms, and pathophysiology of Behçet syndrome

Recurrent aphthous ulcers (canker sores)
Genital ulcers
Uveitis (inflammation of the uvea, middle layer of the eye)
Retinal vasculitis (inflammation of the retina)
Arthritis
Gut lesions
Nervous system problems
Vascular lesions (aneurysms, thrombosis)

Genital manifestations of Behçet syndrome

Genital ulcers appear in the vulva and vagina, and are painful, recurrent, and leave scars. These usually occur less than oral ulcers, but genital ulcers are deeper and larger than mouth ulcers. The vagina can harbour undiscovered asymptomatic ulcers.

Other symptoms of Behçet syndrome

These include oral ulcers, which are the hallmark of the disease. Next in line are the eyes, skin, arthritis, intestinal disturbances, headache, stiff neck, and vascular complaints.

Cause of Behçet disease

There are links with genetic and environmental factors, but no single cause that accompanies every case can be found.

The HLA-B51 allele is often found in patients from Asia and the Middle East, but not in Europeans or Americans. Infection with viruses can be singled out, particularly herpes simplex virus (HSV), hepatitis C, and parvovirus B19.

Streptococcal antigens have also been suspected – prophylactic penicillin decreased acute arthritis episodes in some patients.

If you have a parent with Behcet syndrome, onset tends to be at a younger age. Children with Behçet syndrome are likely to have a family history of the disease.

What else could it be?

Crohn’s disease
Addison’s disease (adrenal insufficiency)
Herpes simplex virus (HSV)
Paediatric sarcoidosis
Lupus
Colitis

Treatment of Behçet syndrome

Each patient will be managed by their physician based on their circumstances. Drugs are used to manage the condition, but there is no cure. Surgery may be used in certain instances.⁶

References

Ehrlich GE. Diagnostic criteria for Behcet’s disease. J Rheumatol. 2000 Aug. 27(8):2049-50. [Medline].
Kim ES, Chung WC, Lee KM, Lee BI, Choi H, Han SW. A case of intestinal Behcet’s disease similar to Crohn’s colitis. J Korean Med Sci. 2007 Oct. 22(5):918-22. [Medline].

1.
Mangelsdorf HC, White WL, Jorizzo JL. Behçet’s disease. Journal of the American Academy of Dermatology. Published online May 1996:745-750. doi:10.1016/s0190-9622(96)90006-7
2.
Alibaz-Oner F, Direskeneli H. Update on the Diagnosis of Behçet’s Disease. Diagnostics. Published online December 23, 2022:41. doi:10.3390/diagnostics13010041
3.
Sula. Demographical and Clinical Characteristics of Behcet’s Disease in Southeastern Turkey. J Clin Med Res. Published online 2014. doi:10.14740/jocmr1952w
4.
Azizlerli G, Akdağ Köse A, Sarıca R, et al. Prevalence of Behçet’s disease in Istanbul, Turkey. Int J Dermatology. Published online October 2003:803-806. doi:10.1046/j.1365-4362.2003.01893.x
5.
Melikoğlu M, Melikoğlu M. The Influence of Age on Behcet’s Disease Activity. Eurasian J Med. 2008;40(2):68-71. https://www.ncbi.nlm.nih.gov/pubmed/25610030
6.
Cobellis L, Pecori E, Rigatti F, et al. Therapeutic alternatives in Behçet’s syndrome. Clin Exp Obstet Gynecol. 2007;34(3):151-153. https://www.ncbi.nlm.nih.gov/pubmed/17937089