Behçet's disease (pronounced beh-CHETS) is a rare inflammatory disorder that causes chronic inflammation in blood vessels throughout the body. This multisystem condition is characterized by recurrent oral and genital ulcers, eye inflammation, and various other symptoms that can affect nearly any part of the body.

Named after the Turkish dermatologist Hulusi Behçet who first described it in 1937, this condition has a worldwide distribution but is more common along the ancient Silk Road, which extends from eastern Asia to the Mediterranean. While relatively uncommon in the United States and Western Europe, understanding Behçet's disease is crucial for timely diagnosis and management of this potentially serious condition.

Causes and Risk Factors

The exact cause of Behçet's disease remains unknown, but research suggests it likely results from a combination of factors:

Autoimmune Mechanisms: The immune system mistakenly attacks healthy cells, particularly blood vessels, leading to inflammation throughout the body.

Genetic Predisposition: The HLA-B51 gene is strongly associated with Behçet's disease, particularly in populations from the Middle East and Asia. Family clusters of the disease suggest inherited susceptibility.

Environmental Triggers: Researchers suspect that certain environmental factors may trigger the disease in genetically predisposed individuals:

Viral or bacterial infections (particularly certain herpes viruses and streptococcal bacteria)

Abnormal immune responses to oral bacteria

Geographic Factors: Prevalence varies significantly by region:

 Highest rates occur in countries along the ancient Silk Road trading route, including Turkey (where prevalence may reach 370 per 100,000 people), Iran, and Japan

 Much lower rates in Western countries (approximately 0.12-0.33 per 100,000 in the U.S. and Northern Europe)

Demographic Patterns:

• Typically develops between ages 20-40

• Can affect both men and women, though severity may differ (men often experience more severe symptoms)

Common Symptoms

Behçet's disease manifests with diverse symptoms that typically come and go in unpredictable flares. The hallmark symptoms include:

Oral Ulcers:

• Painful sores resembling canker sores in the mouth

• May appear on lips, tongue, gums, or inside cheeks

• Usually heal within 1-3 weeks but tend to recur

• Present in nearly all patients and often the first symptom

Genital Ulcers:

• Similar to oral ulcers but may be deeper and more painful

• May leave scars after healing

• Occur in 60-80% of patients

Eye Inflammation (Uveitis):

• Redness, pain, and blurred vision

• Sensitivity to light

• Tearing or eye discharge

• Can lead to vision loss if untreated

• Affects about 50% of patients

Skin Lesions:

• Acne-like bumps

• Erythema nodosum (painful red nodules typically on the legs)

• Superficial thrombophlebitis (inflammation of veins)

Joint Symptoms:

• Pain, swelling, and stiffness in joints

• Most commonly affects knees, ankles, wrists, and elbows

• Usually non-erosive (doesn't damage joints permanently)

Vascular Involvement:

• Inflammation of blood vessels (vasculitis)

• Can affect arteries and veins of all sizes

• May lead to blood clots, aneurysms, or varices Neurological Symptoms (Neuro-Behçet's):

• Headaches

• Meningitis (inflammation of the brain lining)

• Difficulty with balance or coordination

• Cognitive changes

• Occurs in approximately 10-20% of patients

Gastrointestinal Symptoms:

• Abdominal pain

• Diarrhea (sometimes bloody)

• Ulcers in the digestive tract

• More common in patients from Japan and Korea

Diagnosis Methods

Diagnosing Behçet's disease presents challenges due to the lack of a single definitive test and its symptom overlap with other conditions. Diagnosis typically relies on:

Clinical Criteria: The International Study Group for Behçet's Disease established criteria requiring:

Recurrent oral ulcers (at least three times in 12 months), plus at least two of the following:

• Recurrent genital ulceration

• Eye lesions (uveitis or retinal vasculitis)

• Skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions)

• Positive pathergy test (skin hypersensitivity)

Medical History and Physical Examination: Thorough documentation of symptoms and their recurrence patterns is essential.

Pathergy Test: A sterile needle is used to prick the skin; a red bump developing within 24-48 hours suggests Behçet's disease. This test is more sensitive in patients from the Middle East and Far East.

Blood Tests: May show elevated inflammatory markers (ESR, CRP) but are not specific to Behçet's.

Imaging Studies:

• MRI for suspected neurological involvement

• Angiography to assess vascular complications

• Endoscopy for gastrointestinal symptoms

Eye Examination: Complete ophthalmologic assessment to detect uveitis or retinal vasculitis.

Diagnosis often takes time and may require evaluation by multiple specialists, including rheumatologists, dermatologists, ophthalmologists, and neurologists.

Treatment Options

Treatment for Behçet's disease aims to relieve symptoms, reduce inflammation, prevent serious complications, and control the immune system. Approaches include:

Medications:

1.  Topical Treatments:

Corticosteroid mouthwashes or creams for ulcers

Analgesic preparations to relieve pain

2.  Systemic Medications:

Colchicine: Often used for mucocutaneous symptoms

Corticosteroids: For acute flares and eye inflammation

Immunosuppressants: Including azathioprine, methotrexate, cyclosporine

Biologics: TNF inhibitors (infliximab, adalimumab) for severe or resistant cases

Apremilast: A newer option for oral ulcers

Thalidomide: Sometimes used for resistant mucocutaneous lesions (strictly controlled due to severe birth defect risk)

3.  Anticoagulants: For patients with vascular involvement and clotting tendencies

Treatment Strategy:

• Therapy is tailored to organ involvement and symptom severity

• More aggressive treatment for potentially sight-threatening or life-threatening manifestations

• Long-term maintenance therapy is often required

• Regular monitoring for medication side effects

Supportive Care:

• Pain management

• Physical therapy for joint symptoms

• Psychological support for coping with chronic disease

Prevention and Management Tips

While Behçet's disease cannot be prevented entirely, these strategies help manage the condition:

 Avoid Triggers: Identify and avoid personal triggers that may precede flares (stress, certain foods, extreme fatigue)

Oral Care:

• Maintain excellent oral hygiene to minimize complications from mouth sores Use soft toothbrushes
• Avoid spicy, acidic, or rough-textured foods during flares
• Rinse with salt water or prescribed mouthwashes

Eye Care:

• Attend regular ophthalmology check-ups, even when asymptomatic
• Wear sunglasses to reduce photosensitivity
• Seek immediate care for new eye symptoms

Skin Protection:

• Use gentle, fragrance-free skin products
• Avoid tight clothing that may irritate skin lesions

Stress Management:

• Practice relaxation techniques such as meditation or deep breathing
• Maintain healthy sleep patterns
• Consider counseling if needed

Regular Exercise:

• Engage in appropriate physical activity when not experiencing active joint symptoms

Support Network:

• Connect with support groups specific to Behçet's disease

Medication Adherence:

• Take medications as prescribed, even during symptom-free periods

When to See a Doctor

Contact your healthcare provider if you experience:

• Recurrent mouth sores that don't heal within two weeks

• Unexplained genital sores

• Any visual disturbances, eye pain, or redness

• Joint pain accompanied by other Behçet's symptoms

• Symptoms that worsen or don't improve with prescribed treatment

• New symptoms that may be related to Behçet's disease

• Side effects from medications used to treat the condition

If you've been diagnosed with Behçet's disease, maintain regular follow-up appointments with your healthcare team, which may include rheumatologists, dermatologists, ophthalmologists, and other specialists as needed.

Early intervention, particularly for eye involvement, can prevent serious complications and improve long term outcomes.

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Disclaimer

This article is for informational purposes only and does not substitute professional medical advice. Always consult with qualified healthcare providers regarding any medical conditions or symptoms. The information provided here should not be used for diagnosis or treatment of health problems or as a substitute for specialized medical care.