Behcet’s Syndrome

Behcet’s Syndrome is a disorder where an Inflammation process occurs within the body that specifically affects the blood vessels throughout the body.

Symptoms however can affect different parts of the body. Especially the skin, mouth, and Joint.

The cause of Behcet’s syndrome is believed to be Autoimmune. This means that the body will inadvertently attack itself. There are likely other factors such as environment and genetic that play roles as well.

Research is looking into a possible virus that may trigger certain people with a gene that might make them more susceptible.

Men and Women are both affected but often men have more severe symptoms.

Risks:

1.)  Age – most commonly affects men and women in 20’s and 30’s. Can affect children and older adults as well.

2.)  Where you live:  Most often seen in those from the Middle East and East Asia. This includes: Turkey, Iran, Japan, China and more

Potential Symptoms:

1.)  Mouth
–  Painful mouth sores that appear like canker sores
–  Raised, round lesions in the mouth that turn to ulcers
–  Sore take 1-3 weeks to heal
–  They are often reoccurring

2.)  Skin
–  Some develop Acne like sores on the body
–  Some develop red, raised and tender nodules on skin – especially on lower legs

3.)  Genitals
–  Red and open sores on scrotum or the vulva
–  Sore are painful and scars do occur

4.)  Eyes
–  Inflammation in eyes – Also called Uveitis
–  Redness, pain, and blurred vision – usually in both eyes
–  Symptoms can come and go

5.)  Joints
–  Joint swelling and pain
–  Joint often affected is knee
–  The ankles, elbows and wrists my also be involved
–  Swelling and pain may last x 1 -3 weeks and go away on its own

6.)  Blood Vessels
–  Inflammation of veins and arteries
–  Redness, pain, swelling from a blood clot
–  Larger arteries can lead to aneurysms
–  Blockage or narrowing of blood vessels are seen

7.)  Digestive System
–  Abdominal pain
–  Nausea
–  Vomiting
–  Diarrhea
–  Blood in stool
–  Others

8.) Brain
–  Headache
–  Fever
–  Confusion
–  Disorientation
–  Dizziness
–  Poor Balance
–  Stroke

Complications

1.) Blindness

2.) Blood Clots

3.) Strokes

4.) Others

Diagnosis

–  No Blood tests can specifically help with diagnosis
–  Physical Exams
–  Review of Symptoms
–  Mouth Sores – (3 or more times in 12 months) + either genital sores (reoccurring), Eye inflammation, or skin sores
–  Blood work can be important though to help rule out other things
–  Pathergy Test – needle into skin and follow up a few days later. If positive, a small red bump forms under your skin where the needle is inserted.

Treatment

1.)  No cure for Behcet’s Syndrome

2.)  Pain medications

3.)  Medication for inflammation

4.)  Topical creams, gels, ointments

–  Steroid cream – reduces inflammation

5.)  Mouth rinses

–  Rinses with steroids and other agents

6.)  Eyedrops

–  Anti-inflammatory and Corticosteroids

7.)  Systemic Treatment

–  Cochicine  (Colcrys, Mitigare) – for genital and oral sores. Almost might help with joint pains
–  Oral Prednisone
–  Azathioprine (Azasan, Imuran)
–  Cyclosporine (Gengraf, Neoral, Sandimmune)
–  Cyclophosphamide
–  Interferon Aflfa-2b  (Intron A)
–  Tumor necrosis factor (TNF) – Infliximab (Remicade) and Adalimumab (Humira)