is an autoimmune issue
MOST COMMON SYMPTOMS AND SIGNS OF BEHCET'S
Behçet's disease is
common in the Middle East, Asia, and Japan. It is rare in the
United States. In Middle Eastern and Asian countries, the
disease affects more men than women. In the United States, it
affects more women than men. Behçet's disease tends to develop
in people in their 20's or 30's, but people of all ages can
develop this disease.
Behçet's disease is an
autoimmune disease that results from damage to blood vessels
throughout the body, particularly veins.
In an autoimmune
disease, the immune system attacks and harms the bodies' own
tissues. The exact cause of Behçet's disease is unknown.
of the disease are caused by vasculitis (an inflammation of the
Inflammation is a
characteristic reaction of the body to injury or disease and is
marked by four signs: swelling, redness, heat, and pain. Doctors
think that an autoimmune reaction may cause the blood vessels to
become inflamed, but they do not know what triggers this
reaction. Under normal conditions, the immune system protects
the body from diseases and infections by killing harmful
"foreign" substances, such as germs, that enter the body. In an
autoimmune reaction, the immune system mistakenly attacks and
harms the body's own tissues. Behçet's disease is not
contagious; it is not spread from one person to another.
Behçet's disease affects each person differently. The four most
common symptoms (as listed) are mouth sores, genital sores,
inflammation inside of the eye, and skin problems. Inflammation
inside of the eye (uveitis, retinitis, and iritis) occurs in
more that half of those with Behçet's disease and can cause
blurred vision, pain, and redness. Other symptoms may include
arthritis, blood clots, and inflammation in the central nervous
system and digestive organs.
Behcet's disease has the ability to involve blood vessels of
nearly all sizes and types, ranging from small arteries to large
ones, and involving veins as well as arteries. Because of the
diversity of blood vessels it affects, manifestations of
Behcet's may occur at many sites throughout the body. However,
the disease does seem to target certain organs and tissues;
these are described below:
cause either anterior uveitis (inflammation in the
front of the eye) or posterior uveitis (inflammation
in the back of the eye), and sometimes causes both
at the same time.
uveitis results in pain, blurry vision, light
sensitivity, tearing, or redness of the eye.
uveitis may be more dangerous and visionthreatening
because it often causes fewer symptoms while
damaging a crucial part of the eye the retina.
in the mouth called "aphthous ulcers" (known as oral
aphthosis [af-THO-sis] and aphthous stomatitis)
affect almost all patients with Behçet's disease.
Individual sores or ulcers are usually identical to
canker sores, which are common in many people. These
sores are usually a result of minor trauma. They are
often the first symptom that a person notices and
may occur long before any other symptoms appear.
However, the lesions are more numerous, more
frequent, and often larger and more painful.
Aphthous ulcers can be found on the lips, tongue,
and inside of the cheek. Aphthous ulcers may occur
singly or in clusters, but occur in virtually all
patients with Behcet's. The sores usually have a red
border and several may appear at the same time. They
may be painful and can make eating difficult. Mouth
sores go away in 10 to 14 days but often come back.
Small sores usually heal without scarring, but
larger sores may scar.
are a common symptom of Behçet's disease. Skin sores
often look red or resemble pus-filled bumps or a
bruise. The sores are red and raised, and typically
appear on the legs and on the upper torso. In some
people, sores or lesions may appear when the skin is
scratched or pricked. When doctors suspect that a
person has Behçet's disease, they may perform a
pathergy test, in which they prick the skin with a
small needle; 1 to 2 days after the test, people
with Behçet's disease may develop a red bump where
the doctor pricked the skin. However, only half of
the Behçet's patients in Middle Eastern countries
and Japan have this reaction. It is less commonly
observed in patients from the United States, but if
this reaction occurs, then Behçet's disease is
lesions that resemble acne, but can occur nearly
anywhere on the body. This rash is sometimes called
called erythema nodosum: red, tender nodules that
usually occur on the legs and ankles but also appear
sometimes on the face, neck, or arms. Unlike
erythema nodosum associated with other diseases
(which heal without scars), the lesions of Behcet's
disease frequently ulcerate.
"arthralgias" which is inflammation of the joints,
occurs in more than half of all patients with
Behçet's disease. Arthritis causes pain, swelling,
and stiffness in the joints, especially in the
knees, ankles, wrists, and elbows. Arthritis that
results from Behçet's disease usually lasts a few
weeks and does not cause permanent damage to the
disease affects the central nervous system in about
23 percent of all patients with the disease in the
United States. The central nervous system includes
the brain and spinal cord. Its function is to
process information and coordinate thinking,
behavior, sensation, and movement. Behçet's disease
can cause inflammation of the brain and the thin
membrane that covers and protects the brain and
spinal cord. This condition is called
meningoencephalitis. People with meningoencephalitis
may have fever, headache, stiff neck, and difficulty
coordinating movement, and should report any of
these symptoms to their doctor immediately. If this
condition is left untreated, a stroke (blockage or
rupture of blood vessels in the brain) can result.
nervous system involvement is one of the most
dangerous manifestations of Behcet's. The disease
tends to involve the "white matter" portion of the
brain and brainstem, and may lead to headaches,
confusion, strokes, personality changes, and
(rarely) dementia. Behcet's may also involve the
protective layers around the brain (the meninges),
leading to meningitis. Because the meningitis of
Behcet's disease is not associated with any known
infection, it is often referred to as "aseptic"
affect more than half of all people with Behçet's
disease. The sores look similar to the mouth sores
and may be painful. After several outbreaks, they
may cause scarring.
painful genital lesions that form on the scrotum,
similar to oral lesions, but deeper.
painful genital ulcers that develop on the vulva.
disease causes inflammation and ulceration (sores)
throughout the digestive tract that are identical to
the aphthous lesions in the mouth and genital area.
may occur anywhere in the gastrointestinal tract
from the mouth to the anus. This leads to abdominal
pain, diarrhea, and/or bleeding. Because these
symptoms are very similar to symptoms of other
diseases of the digestive tract, such as ulcerative
colitis and Crohn's disease, careful evaluation is
essential to rule out these other diseases.
Above is a colonoscopy of a Behcet's Disease Patient
- showing ulcers.
Prepared by NINDS. Credit goes to the NINDS
and the NIH. Pictures courtesy of Johns Hopkins University
on behalf of its Division of Rheumatology & ABDA. Copyrights