Behcet’s Disease by Dr. Humeira Badsha

What Is It?
Behçet’s Disease (beh-CHETTS) disease is a rare and chronic, life-long condition that affects the inner lining of the mouth and genitals and the small blood vessels throughout the body.
Its symptoms were first written about more than 2,000 years ago by the physician Hippocrates. But it wasn’t until 1937 that Turkish dermatologist Dr. Hulushi Behcet correctly identified the disease.
Who Gets it?
Behcet’s Disease tends to strike people with “Silk Route” bloodlines more frequently than it affects people of other ancestries. Silk Route countries include those in the Mediterranean basin, Middle East and Far East. However, cases of Behcet’s Disease (or Syndrome) have been found worldwide, regardless of patients’ backgrounds.
In the Silk Route countries, Behcet’s Disease is more common in men than in women. However, in Western Europe and the U.S., the trend is reversed: more women have Behçet’s Disease than men.
It occurs primarily in young adults in their 20s and 30s. However, other age groups can also be affected.
Symptoms of Behcet’s Disease
The location of the inflammation(s) of the blood vessel(s) is what dictates your symptoms. Symptoms include recurrent mouth ulcerations, resembling canker sores, recurrent genital ulcers, eye lesions, skin lesions, abdominal pain, swelling of veins in the leg, fatigue and fever.
Behcet’s is a very painful disease. Pain is caused by the ulcers as they come and go, open and heal, year after year.
People with Behcet’s Disease also suffer from arthritis. Behcet’s has the potential to involve all organs, including the heart, lungs and/or bowels. Rarely, it may affect the central nervous system, causing impaired speech, balance, movement and memory loss.
It is chronic, which means it lasts a long time and won’t go away.
What Causes It?
The cause is unknown, but Behcet’s Disease is not infectious, contagious or sexually transmitted.
Researchers believe that disease sufferers may have a genetic predisposition that is triggered by a bacteria or similar cause. People with Behcet’s Disease are often found to have defects in their immune system, the body’s natural defense against bacteria and viruses.

How Is It Diagnosed?
Behcet’s Disease is quite rare and diagnosis is very difficult. Diagnosis is made by eliminating other possible causes. There is no laboratory test, the diagnosis is clinical, based upon symptoms.
Some of the symptoms of Behcet’s Disease also appear in other diseases. It may be necessary to undergo various blood tests and/or biopsies in order to rule them out before you can be diagnosed with Behcet’s.
A person is diagnosed with the condition if mouth ulcers have occurred at least 3 times during a 12-month period.
Plus two of the following:

Genital ulcers (ulcers or sores in the private parts)
Uveitis or retinal vasculitis (inflammation of the eye)
Reddish bumps, pimples, or sores on skin
A pathergy reaction skin test positive. ( abump on the skin if poked by a needle)

Occasionally patients have a positive HLA blood test.
Treatment Options

Because the cause of Behcet’s Disease is unknown, treatment is aimed toward individual symptoms as they occur.
Medication is given to reduce inflammation or regulate the immune system.
The most effect treatment – and the cure – will ultimately depend on the outcome of research being conducted by concerned groups throughout the world.

Medications:

Corticosteroid pills/creams
Pain-relieving mouth rinses
NSAIDs
Colchicine
Cytotoxic drugs
Cyclosporine
Remicade intravenously
Rest and exercise

This is one of a set of articles by Dr. Humeira Badsha, Specialist Rheumatologist, for patient awareness.

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