Behçet’s Disease: Overview, Symptoms, Causes, Diagnosis, and Treatment

A comprehensive medical guide to Behçet’s disease, covering symptoms, causes, diagnosis, treatment options, and risk factors. Learn how this rare inflammatory condition affects the body and how it is managed.

Keywords

Behçet’s disease, Behçet’s syndrome, causes of Behçet’s disease, Behçet’s treatment, oral ulcers, uveitis, inflammation of blood vessels, autoimmune disorders, Behçet’s symptoms, rare diseases

Overview

Behçet’s disease (Behçet’s syndrome) is a rare chronic condition characterized by blood vessel inflammation throughout the body. Early symptoms may appear unrelated, such as painful oral or genital ulcers, skin lesions, eye inflammation, and joint pain.
Treatment aims to relieve symptoms, reduce inflammation, and prevent complications like vision loss.

Symptoms of Behçet’s Disease

Symptoms vary widely and may come and go. They depend on the organs affected.

1. Mouth

Painful mouth ulcers resembling canker sores
Start as raised lesions and develop into painful ulcers
Heal within 1–3 weeks but tend to recur

2. Skin

Acne-like lesions
Painful raised nodules (erythema nodosum), especially on the lower legs

3. Genitals

Painful open sores on the scrotum or vulva
May leave scars after healing

4. Eyes

Redness, pain, blurred vision
Uveitis affecting one or both eyes
Symptoms may flare and subside

5. Joints

Swelling and pain in knees, ankles, elbows, or wrists
Episodes last 1–3 weeks

6. Blood Vessels

Vein and artery inflammation
Pain, redness, blood clots
May cause serious complications like aneurysms

7. Digestive System

Abdominal pain
Diarrhea
Gastrointestinal bleeding

8. Nervous System

Headaches, fever
Confusion
Balance problems
Possible stroke in severe cases

When to See a Doctor

Seek medical attention if you develop symptoms of Behçet’s disease.
If already diagnosed, consult your doctor when new symptoms appear.

Causes

Behçet’s disease is believed to be an autoimmune disorder in which the immune system attacks the body’s own tissues.
Possible contributing factors include:

Genetic predisposition
Environmental triggers
Infections in genetically susceptible individuals

Vasculitis (inflammation of blood vessels) is the underlying mechanism.

Risk Factors

People are more likely to develop Behçet’s disease if they have:

Age: Most common between ages 20–40
Geography: Higher rates in the Middle East and East Asia
Sex: More severe in males
Genetics: Certain gene types increase risk

Complications

Complications vary depending on affected organs.
Untreated eye inflammation (uveitis) can lead to irreversible vision loss or blindness.
Regular eye examinations are essential for early management.

Diagnosis

There is no single test for Behçet’s disease. Diagnosis is based mainly on clinical symptoms.

Diagnostic Criteria

Recurrent mouth ulcers (3+ times within 12 months)
At least two of the following:
- Recurrent genital ulcers
- Eye inflammation
- Skin lesions

Supporting Tests

Blood tests: To rule out other conditions
Pathergy test: A small raised bump after needle insertion indicates immune overreaction

Treatment

Although there is no cure, treatments help manage inflammation and prevent flare-ups.

Medications for Acute Flare-Ups

Topical corticosteroids: Creams and gels for skin and genital ulcers
Medicated mouthwash: Steroid-based solutions for oral ulcers
Corticosteroid eye drops: For mild eye inflammation

Systemic Treatments

Used when symptoms are severe or widespread:

1. Colchicine

Helps reduce mouth/genital ulcers and joint inflammation

2. Apremilast (Otezla)

FDA-approved for oral ulcers
Side effects: weight loss, depression

3. Corticosteroids (e.g., Prednisone)

Reduce body-wide inflammation
Often combined with immunosuppressants
Side effects: weight gain, hypertension, heartburn, osteoporosis

4. Immunosuppressants

Azathioprine, cyclosporine, cyclophosphamide
Risks: infections, liver/kidney issues, blood cell reduction

5. Biologic Therapies

TNF inhibitors: Infliximab (Remicade), Adalimumab (Humira)
For severe or refractory symptoms
Side effects: rash, headache, increased infection risk

Questions Your Doctor May Ask

Are your symptoms persistent or episodic?
What improves your symptoms?
What triggers worsening symptoms?
Does anyone in your family have Behçet’s disease?

Behçet’s Disease