Behcet's Syndrome
Medical Author: William C. Shiel Jr., MD, FACP, FACR
What is Behcet's syndrome?
Behcet's syndrome is classically characterized as a triad of
symptoms
that include recurring crops of mouth ulcers (aphthous
ulcers),
genital ulcers, and inflammation of a specialized area around
the pupil of
the eye (the uvea). The inflammation of the area of the eye that
is around
the pupil is called uveitis. Behcet's syndrome is also
sometimes referred
to as Behcet's disease.
The cause of Behcet's syndrome is not known. The disease is
more
frequent and severe in patients from the Eastern Mediterranean
and Asia
than those of European descent. Both inherited (genetic) and
environmental factors, such as microbe infections, are suspected to
be factors that contribute to the development of Behcet's syndrome. Behcet's syndrome is not felt to be contagious.
What are symptoms of Behcet's syndrome?
The symptoms of Behcet's syndrome depend
on the area of the body affected. Behcet's syndrome can involve
inflammation of many areas of the body. These areas include the
arteries
that supply blood to the body's tissues. Behcet's syndrome can
also affect
the veins that take the blood back to the lungs to replenish
the oxygen
content. Other areas of body that can be affected by the
inflammation of
Behcet's syndrome include the back of the eyes (retina), brain,
joints,
skin, and bowels.
The mouth and genital ulcers are generally painful and recur
in crops (many shallow ulcers at the same time).
They range in size from a few millimeters to 20 millimeters in
diameter. The mouth ulcers occur on the gums, tongue, and inner
lining of the mouth. The genital ulcers occur on the scrotum and
penis of males and vulva of women and can leave scars.
Inflammation of the eye, which can involve the front of the
eye (uvea)
causing uveitis, or the back of the eye (retina) causing
retinitis, can
lead to blindness. Symptoms of eye inflammation include pain,
blurred vision, tearing, redness, and pain when looking at bright
lights. It is very important for patients to have
this
sensitive area monitored by an eye specialist (ophthalmologist).
If the arteries become inflamed (arteritis) in patients with
Behcet's
syndrome, it can lead to death of the tissues whose oxygen
supply depends
on these vessels. This could cause a stroke if affecting the
brain, belly
pain if affecting the bowel, etc. When veins become inflamed
(phlebitis),
the inflammation can involve large veins that develop blood
clots which
can loosen to cause pulmonary embolism.
Symptoms of inflammation of the brain or tissue that covers
the brain
(meninges) include headaches, neck stiffness, and is often
associated with
fever. Inflammation of the brain (encephalitis) and/or the meninges (meningitis) can cause damage to
nervous tissue and lead to
weakness or impaired function of portions of the
body. This can result in confusion and coma. Typically these features occur later in the
disease course, years after the diagnosis.
Joint inflammation (arthritis) can lead to swelling,
stiffness, warmth,
pain, and tenderness of joints in patients with Behcet's
syndrome. This occurs in about half of patients with Behcet's
syndrome at sometime during their lives. Knees, wrists, ankles, and
elbows are the most common joints affected.
The skin of patients with Behcet's syndrome can develop areas
of
inflammation which spontaneously appear as raised, tender,
reddish nodules
(erythema nodosum), typically on the front of the legs. Some
patients with
Behcet's syndrome develop a peculiar red or blistery skin
reaction in
places where they have been pierced by blood-drawing needles
(see pathergy test in diagnosis section below).
Recent research has found that acne occurs more frequently in
patients with Behcet's syndrome that also have arthritis as a
manifestation.
Ulcerations can occur at any location in the stomach, large or
small
bowel in patients with Behcet's disease.
Next: How is Behcet's syndrome diagnosed? »
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Last Editorial Review: 2/25/2008
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