7 Key Symptoms of Behcet’s Disease You Should Know

Behcet’s disease, also known as Behcet’s syndrome, is a rare chronic inflammatory disorder that causes blood vessel inflammation throughout the body. Named after Turkish dermatologist Hulusi Behçet who first described it in 1937, this condition can affect multiple organ systems and varies greatly in severity from person to person. Understanding the symptoms is crucial for early detection and management of this complex autoimmune condition.

The symptoms of Behcet’s disease typically appear in episodes or flares, with periods of remission in between. While the condition can affect anyone, it’s most commonly diagnosed in people from Mediterranean countries, the Middle East, and East Asia. Let’s explore the seven key symptoms that characterize this challenging disease.

1. Mouth Sores (Oral Ulcers)

Mouth sores are the hallmark symptom of Behcet’s disease and occur in nearly all patients with this condition. These painful ulcers are often the first sign of the disease and tend to recur frequently.

Characteristics of oral ulcers in Behcet’s disease:

• Appear as round or oval sores with a red border and yellow or white center
• Typically develop inside the mouth, on the tongue, inner cheeks, gums, or throat
• Range in size from small (2-3mm) to large (up to 20mm or more)
• Can appear singly or in clusters of multiple ulcers
• Usually very painful, making eating and drinking difficult
• Generally heal within 1-3 weeks without scarring, though larger ulcers may leave scars
• Recur frequently, sometimes with only brief periods between outbreaks

The pain from these ulcers can significantly impact quality of life, affecting eating, speaking, and oral hygiene. Unlike common canker sores, Behcet’s mouth ulcers tend to be more severe, numerous, and persistent.

2. Genital Sores

Genital ulcers are the second most common symptom of Behcet’s disease, affecting approximately 75-90% of patients. These sores are similar in appearance to oral ulcers but often more painful and may leave scars.

Key features of genital ulcers:

• In men: typically appear on the scrotum, less commonly on the penis
• In women: develop on the vulva, vagina, or cervix
• Appear as painful, deep ulcers with raised red borders
• Often larger and deeper than oral ulcers
• More likely to leave scars after healing
• Can take longer to heal than mouth sores, sometimes 2-4 weeks
• May be accompanied by swelling in the affected area

Genital ulcers can cause significant discomfort during urination, sexual activity, and daily movement. The psychological impact of recurrent genital sores can also be substantial, affecting intimate relationships and self-esteem.

3. Eye Inflammation (Uveitis)

Eye involvement is one of the most serious manifestations of Behcet’s disease, occurring in 30-70% of patients. Ocular symptoms can lead to vision problems and, if left untreated, may result in permanent vision loss.

Types of eye inflammation in Behcet’s disease:

• Uveitis: inflammation of the middle layer of the eye (uvea), the most common eye problem
• Anterior uveitis: affects the front part of the eye, causing redness, pain, and light sensitivity
• Posterior uveitis: affects the back of the eye, potentially more serious as it can damage the retina
• Retinal vasculitis: inflammation of blood vessels in the retina

Common eye symptoms include:

• Eye redness and pain
• Blurred or decreased vision
• Sensitivity to light (photophobia)
• Floaters (spots or threads in vision)
• Tearing or discharge
• Symptoms may affect one or both eyes

Eye inflammation in Behcet’s disease tends to be recurrent and can progressively worsen with each episode if not properly managed. Regular ophthalmologic monitoring is essential for patients with this condition.

4. Skin Lesions

Skin manifestations occur in about 75-80% of people with Behcet’s disease and can take various forms. These skin problems can appear anywhere on the body and may be one of the earlier signs of the condition.

Common types of skin lesions:

• Erythema nodosum-like lesions: tender, red, raised bumps or nodules, typically on the legs
• Pseudofolliculitis: acne-like pustules or papules that resemble infected hair follicles
• Papulopustular lesions: small bumps filled with pus
• Pathergy: an abnormal skin reaction where minor injuries (like needle pricks) cause inflamed bumps or pustules to form

Characteristics of skin lesions:

• May be painful or tender to touch
• Can appear on the face, trunk, arms, or legs
• Often leave hyperpigmentation or darkened spots after healing
• May occur in crops or clusters
• Can be triggered by minor trauma or injury

The pathergy phenomenon, where skin overreacts to minor injuries, is particularly characteristic of Behcet’s disease and is sometimes used as a diagnostic test, though it’s not present in all patients.

5. Joint Pain and Swelling (Arthritis)

Joint involvement affects approximately 50-60% of patients with Behcet’s disease. The arthritis associated with this condition is typically non-erosive, meaning it doesn’t cause permanent joint damage or deformity.

Joint symptoms include:

• Pain and swelling in the joints
• Stiffness, particularly in the morning
• Most commonly affects knees, ankles, wrists, and elbows
• Usually involves large joints more than small joints
• Symptoms may affect one joint (monoarthritis) or multiple joints (polyarthritis)
• Episodes typically last a few weeks and resolve without permanent damage
• Can be migratory, affecting different joints at different times

Impact on daily life:

• Reduced mobility during flares
• Difficulty with walking when lower limb joints are affected
• Challenges with manual tasks when upper limb joints are involved
• Joint symptoms often coincide with other disease flares

While the arthritis of Behcet’s disease doesn’t cause permanent joint destruction like rheumatoid arthritis, the recurrent nature of symptoms can significantly impact physical activity and quality of life.

6. Vascular Inflammation (Vasculitis)

Blood vessel inflammation is a defining feature of Behcet’s disease, though symptomatic vascular involvement occurs in about 10-30% of patients. This symptom can affect both arteries and veins of various sizes throughout the body.

Venous manifestations:

• Superficial thrombophlebitis: inflammation of veins near the skin surface, causing red, tender cords along the vein path
• Deep vein thrombosis (DVT): blood clots in deep veins, most commonly in the legs
• Symptoms: leg swelling, pain, warmth, and redness
• Can affect major veins including the vena cava

Arterial manifestations (less common but more serious):

• Aneurysms (bulging in artery walls)
• Arterial thrombosis (blood clots in arteries)
• Arterial narrowing or blockage
• Most commonly affects pulmonary arteries, aorta, and peripheral arteries

Warning signs of vascular involvement:

• Swelling in arms or legs
• Persistent leg pain or cramping
• Chest pain or shortness of breath
• Sudden severe headache
• Abdominal pain

Vascular complications are among the most serious aspects of Behcet’s disease and can be life-threatening. Prompt medical attention is necessary if these symptoms develop.

7. Neurological Symptoms

Central nervous system involvement, known as neuro-Behcet’s disease, occurs in approximately 5-10% of patients but represents one of the most serious complications. Neurological symptoms can develop years after other symptoms appear or, less commonly, be the initial presentation.

Two main patterns of nervous system involvement:

Parenchymal neuro-Behcet’s (most common):

• Affects the brain tissue itself
• Headaches (often severe and persistent)
• Confusion or cognitive difficulties
• Behavioral or personality changes
• Speech difficulties
• Weakness or numbness on one side of the body
• Difficulty with coordination and balance
• Seizures

Non-parenchymal neuro-Behcet’s:

• Involves blood vessels in the brain
• Cerebral venous thrombosis (blood clots in brain veins)
• Severe headaches
• Vision problems
• Increased intracranial pressure

Additional neurological manifestations:

• Aseptic meningitis (inflammation of brain membranes)
• Memory problems
• Depression and mood changes
• Difficulty concentrating
• Vertigo or dizziness

Neurological involvement can significantly impact long-term prognosis and requires aggressive management. Any new neurological symptoms in a patient with Behcet’s disease warrant immediate medical evaluation.

Main Causes of Behcet’s Disease

The exact cause of Behcet’s disease remains unknown, but research suggests it results from a combination of genetic, environmental, and immunological factors. Understanding these potential causes helps explain why this condition develops.

Genetic Factors:

• HLA-B51 gene: The strongest genetic association with Behcet’s disease; people with this gene variant have a higher risk, though not everyone with HLA-B51 develops the disease
• Family history increases risk, suggesting hereditary components
• More common in people from the Mediterranean region, Middle East, and East Asia (along the historic Silk Road)
• Affects men and women, though severity may differ between genders

Autoimmune Dysfunction:

• The immune system mistakenly attacks healthy blood vessels throughout the body
• Abnormal T-cell and neutrophil activity contributes to inflammation
• Excessive inflammatory response to normal triggers
• Loss of regulation in immune system function

Environmental Triggers:

• Infections: Certain bacterial or viral infections may trigger disease onset in genetically susceptible individuals
  • Streptococcal bacteria have been implicated
  • Herpes simplex virus has been studied as a potential trigger
• Geographic location: Higher prevalence in specific regions suggests environmental factors
• Stress: May trigger flares in those already diagnosed

Other Contributing Factors:

• Age: Most commonly diagnosed in people in their 20s and 30s
• Gender differences: Men often experience more severe symptoms, particularly eye and vascular involvement
• Abnormal response to minor trauma (pathergy)

Behcet’s disease is classified as an autoinflammatory disorder rather than a purely autoimmune disease, as it involves innate immune system dysfunction without the typical autoantibodies seen in classic autoimmune conditions.

Frequently Asked Questions

What is Behcet’s disease?

Behcet’s disease is a rare chronic inflammatory disorder that causes blood vessel inflammation throughout the body. It’s characterized by recurrent oral and genital ulcers, eye inflammation, skin lesions, and can affect joints, blood vessels, the nervous system, and digestive tract.

Is Behcet’s disease contagious?

No, Behcet’s disease is not contagious. You cannot catch it from or spread it to another person. It’s an autoinflammatory condition caused by immune system dysfunction.

How is Behcet’s disease diagnosed?

There’s no single test for Behcet’s disease. Diagnosis is based on clinical criteria, including recurrent oral ulcers plus at least two of the following: recurrent genital ulcers, eye inflammation, skin lesions, or a positive pathergy test. Doctors also rule out other conditions with similar symptoms.

Can Behcet’s disease be cured?

Currently, there is no cure for Behcet’s disease. However, symptoms can be managed with appropriate medical care, and many patients experience periods of remission where symptoms are minimal or absent.

Who is most at risk for Behcet’s disease?

Behcet’s disease is most common in people from Mediterranean countries, the Middle East, and East Asia, particularly those between ages 20-40. People with the HLA-B51 gene variant have higher risk, and having a family history increases susceptibility.

Does Behcet’s disease affect life expectancy?

Most people with Behcet’s disease have a normal life expectancy. However, serious complications involving the nervous system, major blood vessels, or eyes can impact long-term prognosis. Regular medical monitoring and appropriate management are essential.

Can Behcet’s disease symptoms come and go?

Yes, Behcet’s disease typically follows a relapsing-remitting pattern with flares of active symptoms followed by periods of remission. The frequency, severity, and duration of flares vary greatly between individuals.

What triggers a Behcet’s disease flare?

Triggers vary by individual but may include stress, infections, certain foods, hormonal changes, fatigue, extreme temperatures, or minor injuries. Identifying and avoiding personal triggers can help reduce flare frequency.

Should I see a doctor if I have recurring mouth sores?

Yes, if you experience frequent, painful mouth sores along with other symptoms like genital sores, eye problems, or unusual skin lesions, you should consult a healthcare provider for evaluation. Early diagnosis and management can help prevent complications.

Can pregnancy affect Behcet’s disease?

Pregnancy can affect Behcet’s disease differently in different women. Some experience improvement in symptoms during pregnancy, while others may have flares. Women with Behcet’s disease should work closely with their healthcare team before and during pregnancy for optimal management.

References:

• Mayo Clinic – Behcet’s Disease
• Johns Hopkins Medicine – Behcet’s Disease
• NHS – Behcet’s Disease
• National Organization for Rare Disorders – Behcet’s Disease
• National Institute of Arthritis and Musculoskeletal and Skin Diseases – Behcet’s Disease