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‫) ‪Behçet's disease ( BD‬‬

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‫محمد علي حريج كشكول المرحلة ‪ :‬الرابعة ‪ -‬مسائي‬
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Behçet's disease ( BD )
 Introduction

is a type of inflammatory disorder which affects multiple parts of the

body.The most common symptoms include painful mouth sores, genital
sores, inflammation of parts of the eye, and arthritis.The sores typically
last a few days.Less commonly there may be inflammation of the brain
or spinal cord, blood clots, aneurysms, or blindness.Often the
symptoms come and go. The cause is unknown.It is believed to be
partly genetic.Behçet's is not contagious.Diagnosis is based on at least
three episodes of mouth sores in a year together with at least two of the
following: genital sores, eye inflammation, skin sores, a positive skin
prick test.
 General Discussion

Behçet's syndrome is a rare multisystem inflammatory disorder

characterized by ulcers affecting the mouth and genitals, various skin
lesions, and abnormalities affecting the eyes. Symptoms include
mucous membrane lesions of the mouth (canker sores) and genitals
(ulcers) that tend to disappear and recur spontaneously. Inflammation
of the eyes (anterior uveitis, posterior uveitis, or panuveitis) also
affects individuals with Behçet's syndrome. Additional systems of
the body may also be affected including the joints, blood vessels,
central nervous system, and/or digestive tract. The exact cause of
Behçet's syndrome is unknown.(1)
 Signs & Symptoms

The earliest symptom of Behçet’s syndrome is usually painful

canker sores on the mucous membranes that line the mouth
(aphthous(
stomatitis). The sores are usually round or oval with reddish
(erythematous) borders that may occur anywhere within the
mouth. They may be shallow or deep and may appear as a
single lesion or a cluster of multiple lesions. The sores typically
heal within a few days, (1)
 Signs & Symptoms

up to a week or more, without scarring, but frequently

recur. They may precede other symptoms of Behçet’s
syndrome by a number of years. Sometimes similar
sores may appear on the genitals, specifically the
scrotum and shaft of the penis in males and the vulva
in females. The sores are also round and painful, but
may be larger and deeper than those affecting the
mouth. These sores also recur,
but unlike oralsores, may tend to scar. (2)
 Signs & Symptoms

Behçet’s syndrome may also affect the eyes. Symptoms

may include inflammation of the back of the eye
(posterior uveitis) and inflammation of the anterior
chamber (anterior uveitis or iridocyclitis).Inflammation
of the iris accompanied by pain, tearing
(lacrimation),and the accumulation of pus (hypopyon
iritis) may also occur.
 Signs & Symptoms

The retina may become inflamed resulting in blurred vision, abnormal

sensitivity to light (photophobia), and/or, inflammation of the thin
membranous layer of blood vessels behind the retina (chorioretinitis).
Although the lesions that cause inflammation in various parts of the
eyes may resolve, repeated recurrences may result in the partial lossof
vision (decreased visual acuity)
or complete blindness if the disease
is uncontrolled. (2)
 Signs & Symptoms

In some cases, eye abnormalities may be the first

symptom of
Behçet’s syndrome. In other cases, they may not
develop until several years later.. Individuals with
Behçet’s syndrome may also exhibit the formation of
small, pus-filled growths on the skin (pustules). Some
affected individuals, especially females, may develop
lesions that resemble those of erythema nodosum,
 Signs & Symptoms

a skin disorder characterized by the formation of tender, reddish,

inflammatory nodules on the front of the legs. These nodules
disappear on their own (spontaneously) sometimes leaving faint scars
or discoloration (pigmentation). Some individuals with Behçet’s
syndrome may develop small eruptions that resemble acne (acneiform
eruptions) and/or inflammation that mistakenly appear to affect the
hair follicles on the skin (pseudofolliculitis(4)
 Signs & Symptoms

In approximately 50 percent of cases of Behçet’s syndrome, affected

individuals experience pain (arthralgia) and swelling in various joints of
the body (polyarthritis). This may occur before, during, or after the
onset of the other symptoms associated with Behçet’s syndrome.
Pain, which can range from mild to severe, typically affects the joints
of the knees, wrists, elbows and ankles, and may become chronic.
Lasting damage to affected joints is extremely rare.
Individuals with Behçet’s syndrome may also have recurring ulcers in
the digestive tract. Symptoms vary from mild abdominal discomfort to
severe inflammation of the large intestine and rectum accompanied by
diarrhea or bleeding.
Approximately 10%-20% of individuals with Behçet’s syndrome also
have involvement of the central nervous system. These symptoms
usually appear months or years after the initial symptoms of Behçet’s
 Signs & Symptoms

syndrome. Recurring attacks of inflammation involving the

brain (parenchymal Neuro-Behçet) or the membranes that
surround the brain or spinal cord (meningitis or
meningoencephalitis) can result in neurological damage.
Symptoms may include headache, the inability to coordinate
voluntary movement (cerebellar ataxia), impaired muscle
movements of the face and throat (pseudobulbar palsies),
stroke, and/or rarely, seizures.
Behçet’s syndrome causes inflammation of the blood vessels
(5)
 Signs & Symptoms

(vasculitis). Involvement of small vessels is thought to drive

many of the problems that the disorder causes. In some
instances,
inflammation of the large veins, particularly those in the legs
may occur along with the formation of blood clots
(thrombophlebitis). The walls of an involved artery may bulge
forming a sac (aneurysm). In very rare cases, blood clots from
the veins travel to the lungs (pulmonary emboli) resulting in
episodes of chest pain, coughing, difficult or labored breathing
(dyspnea), and coughing up blood
(hemoptysis).
 Causes

It is especially important to identify Behçet’s disease when there is

ocular, central nervous system or large blood vessel involvement as
manifestations are usually the most serious.
■Causes
The exact cause of Behçet’s syndrome is not known. Studies suggest
that some people may have a genetic predisposition to the condition.
A genetic predisposition means that a person may carry a gene for a
disease but it may not be expressed unless something in the
environment triggers the disease. Researchers have demonstrated
that certain individuals with Behçet’s syndrome, especially those of
 Causes

Middle Eastern and Asian descent, have an increased frequency of

certain “human leukocyte antigens” (HLAs) in the blood. Individuals
with Behçet’s syndrome are more likely to have HLA-B51 than the
general population. The possible role of HLA-B51 in predisposing
individuals to Behçet’s syndrome and its overall association with the
disorder is unknown. Other genetic markers and their role in the
development of Behçet’s disease are being studied. Viral or bacterial
infections have also been suggested as a possible cause for the
disorder. Still another theory is that the disease is an
auto-inflammatory disorder in which the body loses the ability to
appropriately regulate and control inflammation. (1,4)
 Causes

Autoimmune disorders are caused when the body’s natural defenses

against “foreign” or invading organisms (e.g., antibodies) begin to
attack healthy tissue for unknown reasons. While investigation is
ongoing, no autoantibodies to date have been identified to suggest
that Behçet’s syndrome is an autoimmune disease.
Affected Populations Behçet’s syndrome is a rare disorder in the United States and
Western Europe. It occurs most frequently in the Middle East and
Asia, along ancient trading routes between the Mediterranean basin
and eastern Asia, known as the Silk Road. Turkey has the highest
prevalence rate (80-370 cases per 100,000); Japan, Korea, China,
Iran, and Saudi Arabia also have high prevalence rates. The disorder
is the leading cause of blindness in Japan. The age of onset is
typically between 30 and 40 years.
 Diagnosis

The diagnosis of Behçet’s syndrome is made based on the clinical

judgment of a physician. Criteria have been accepted, based upon the
identification of recurrent oral ulcerations (aphthous stomatitis) that
occur along with at least two of the following: eye lesions, skin lesions,
recurrent genital ulcerations, and a positive pathergy test. (During a
pathergy test, a physician pricks an individual with a sterile needle. A
positive outcome occurs if a reddish spot (nodule or pustule) forms 48
hours after the prick.) However, these criteria have been formed so
that patients might be included in clinical studies (“classification
criteria”) and are not really “diagnostic” criteria. (5)
 Standard Therapies

The treatment of Behçet’s syndrome is directed toward the

specific symptoms that are apparent in each individual.
Specific therapies for Behçet’s syndrome are symptomatic and
supportive. Severity of the condition as well as the patient’s
age and sex may all affect treatment decisions. Spontaneous
remission over time is common for individualswith Behçet’s
syndrome.
 Standard Therapies

For recurrent ulcers, the application of corticosteroid-containing

preparations to the affected areas may be helpful in aborting
developing attacks. Mouthwash containing a local anesthetic such as
Xylocaine, lidocaine, or Benadryl may temporarily relieve pain.
Arthritis associated with Behçet’s syndrome may be treated with
colchicine and nonsteroidal anti-inflammatory drugs (NSAIDs).
Continuing therapy with the drug colchicine may be effective in
preventing recurring attacks of oral and genital ulcers or arthritis.
 Standard Therapies
associated with Behçet’s syndrome. Central nervous system and
vascular abnormalities may be treated with corticosteroids as well,
often in conjunction with immunosuppressive agents. In patients with
clotting of major blood vessels, systemic anticoagulants and
immunosuppressants should be considered.
Inflammation of the joints, skin, and/or mucous membranes or other
organs may be reduced with oral corticosteroid drugs. However,
corticosteroids do not prevent recurring episodes of symptoms and
may not reduce damage when used alone. Therefore,
immunosuppressive agents such as azathioprine, methotrexate,
cyclosporine, or chlorambucil may be employed for improved control
of inflammation and organ protection. Experience is evolving with the
use of interferon-alpha and with agents which inhibit tumor necrosis
factor (TNF) in the treatment of Behçet’s disease. (5)
 References
1. "Behçet's Syndrome" . NORD September 2017.

(National Organization for Rare Disorders). 4. Bolster MB (2009). MKSAP 15 Medical

2015. Archived from Knowledge Self-assessment
the original on 11 February 2017. Retrieved 29 Program: Rheumatology.
May 2017. Philadelphia, Pa: American College of
2. Fleming, Ray (November 2014). "Fast Facts Physicians. ISBN
About Behçet's 978-1-934465-30-1 .
Disease" . www.niams.nih.gov. Archived from 5. Eye (7 January 2011). "Access : A case of
the original on 13 anterior ischemic optic
May 2017. Retrieved 29 May 2017. neuropathy associated with Behçet's disease :
3. Ball, Gene V.; Fessler, Barri J.; Jr, Eye" . Eye.
Bridges (2014). Oxford Textbook of Vasculitis Nature.com. 25 (3): 395–396.
(3 ed.). OUP Oxford. doi : 10.1038/eye.2010.208 .
p. 491. ISBN 9780191667022 . Archived from PMC 3178306 . PMID 21212799 unless
the original on 10 otherwise noted