What is it?
Behçet’s syndrome (BS), or Behçet’s disease, is a systemic vasculitis
(inflammation of blood vessels) of unknown cause. The main symptoms are
recurrent oral and genital ulcers, eye, joint, skin, blood vessel, and nervous
system involvement. BS was named after a Turkish doctor, Prof. Dr. Hulusi
Behçet, who described it in 1937.
How common is it?
BS is more common in some parts of the world. The geographical distribution of
BS coincides with the historical ‘silk route’. It is mainly observed in
countries of the Far East, Middle East and Mediterranean basin, like Japan,
Korea, China, Iran, Turkey, Tunisia, and Morocco. The prevalence rate among the
adult population is 1/10,000 in Japan and 1-3/1,000 in Turkey. The prevalence is
about 1/300.000 in Northern Europe.
Few cases are reported from the United State and Australia. BS in children is
rare, even in high risk populations. The diagnostic criteria are fulfilled,
before the age of 16, in approximately 3% of all BS patients. Overall, the age
of disease onset is 20-35. It is equally distributed amongst both sexes, but
males have a more severe disease.
What are the causes of the disease?
The cause of the disease is unknown. Genetic susceptibility may have some role
in the development of BS. There is no known specific trigger.
Research into the cause and treatment are being carried out in several centres.
Is it inherited?
There is no consistent pattern of inheritance in BS, however some genetic
susceptibility is suspected. The syndrome is associated with a genetic
predisposition (HLA-B5), especially in patients originating from the
Mediterranean and the Far East. There have been reports of cases of families
suffering from this disease.
Why has my child got this disease? Can it be prevented?
The cause of the disease is unknown. BS cannot be prevented.
Is it contagious?
No, it is not.
What are the main symptoms?
1) Oral ulcers: These lesions are almost always present. Oral ulcers are the
initial sign in about two-thirds of patients. The majority of children develop
multiple, minor ulcers, indistinguishable from those seen in recurrent ulcers,
which are common in childhood. Large ulcers are more rare, but can be very
difficult to treat.
2) Genital ulcers: In boys the ulcers are located mainly on the scrotum and,
less frequently, on the penis. In adult male patients, these almost always leave
a scar. The external genitalia are, mainly, affected in girls. These ulcers
resemble the oral ulcers. Children before puberty have less genital ulcers.
Boys may have recurrent orchitis.(testicular inflammation)
3) Skin involvement: There are different skin lesions. Acne-like lesions are
present only after puberty. Erythema nodosum are red, painful, nodular lesions,
usually located on the lower legs. These lesions are more frequent among
Pathergy reaction is the reactivity of the skin of BS patients to a needle
prick. This is used as a diagnostic test in BS. After a skin puncture with a
sterile needle on the forearm, a papule, or pustule forms in 24 to 48 hours.
4) Eye involvement: This is one of the most serious manifestations of the
disease. While the overall prevalence is approximately 50%, it increases to 70%
in boys. Girls are affected less frequently. Disease is bilateral in the
majority of patients. Eyes are involved usually within the first three years
after the disease starts.
The course of the eye disease is chronic, with occasional flares. After each
flare some structural damage occurs, causing gradual vision loss.
5) Joint involvement: Joints are involved in about 30-50% of the children with
BS. Usually ankles, knees, wrists and elbows are affected and less than four
joints are involved. This inflammation usually last a few weeks and resolves on
its own. It is very rare for arthritis of BS to cause joint damage.
6) Neurological involvement: Rarely, children with BS can develop neurological
problems. Seizures, increased intracranial pressure with associated headaches
and cerebral symptoms are characteristic. The most severe forms are seen in
males. Some patients may develop psychiatric problems.
7) Vascular involvement is seen in about 12-30% of juvenile BS patients and can
signal a poor outcome.
Usually the large blood vessels are involved. Commonly effected are the vessels
of the calves, resulting in them becoming swollen and painful.
8) Gastrointestinal involvement: This is especially common in patients from the
Far East. Examination of the bowel reveals ulcers.
Is the disease the same in every child?
No, it is not. Some may have mild disease with oral ulcers and some skin
lesions, others may develop eye or nervous system involvement. There are also
some differences between girls and boys. Boys usually have a more severe disease
course, with more eye and vascular involvement than girls.
Is the disease in children different from the disease in adults?
BS is rare in children compared to adults. There are some differences with
reference to puberty. The disease in post-pubescent children is more like the
adult disease. There are more familial cases among children with BS than adults.
In general, in spite of some variations, BS in children does resembles the adult
How is it diagnosed?
The diagnosis is mainly clinical. It may take one to five years before a child
fulfills the international criteria described for BS. The diagnosis is usually
delayed for an average of three years.
There are no specific laboratory findings for BS. Approximately half of the
children carry HLA-B5 and this is linked to the more severe forms of the
As previously described, a pathergy skin test is positive in about 60% 70 of the
To diagnose vascular and nervous system involvement, specific imaging of the
vessels and the brain may be needed.
Because BS is a multi-system disease, specialists in the treatment of eyes
(ophthalmologist), skin (dermatologist), and the nervous system (neurologist)
are co-operatively involved in the treatment of these patients.
What is the importance of tests?
1) A pathergy skin test is important for diagnosis. It is included in the
International Study Group classification criteria for Behçet’s Disease. Three to
five skin punctures are applied on the inner surface of the forearm with a
sterile needle. It hurts very little and the reaction is evaluated 24 to 48 h
This hyperreactivity can also be seen at sites where blood is drawn, or after
surgery, therefore, patients with BS should not undergo unnecessary
2) Some blood tests are done for differential diagnosis, but there is no
specific laboratory test for BS. Tests that show inflammation in general, are
mildly elevated. A moderate anemia and an increase in white blood cell count may
be detected. There is no need to repeat these tests, unless the patient is being
monitored for disease activity and drug side effects.
3) Several Imaging techniques are applied to children with vascular and
Can it be treatedor cured?
The disease can go into remission, but may have flare-ups. It can be controlled,
but not cured.
What are the treatments?
There is no specific treatment, because the cause of BS is unknown. Different
organ involvement requires different treatment regimes. At one end of the
spectrum are patients with BS who do not need any therapy. On the other hand,
patients with eye, central nervous system and vascular disease may require a
combination of treatments.
Almost all the data available on the treatment of BS come from adult studies.
The main drugs are listed below:
a) Colchicine: Previously, this drug has been used for almost every
manifestation of BS but in a recent study it was shown to be more effective in
the treatment of joint problems and erythema nodosum.
b) Corticosteroids: They are very effective in controlling inflammation.
Steroids are mainly administered to children with eye, central nervous system
and vascular disease, usually in large oral doses (1-2 mg/kg/day). When needed,
they can be also given intravenously in higher doses (30 mg/kg/day administered
as three doses on alternate days), to achieve an immediate response. Topical
(locally administered) steroids are used to treat oral ulcers, and eye disease
in the form of eye drops.
c) Immunosuppressive drugs: This group of drugs are administered to children
with severe disease, especially for eye and major organ involvement. They
include Azathioprin, Cyclosporin-A and Cyclophosphamide.
d) Anti-aggregant and anti-coagulant therapy: used in selected cases with
vascular involvement. In the majority of the patients aspirin is probably
sufficient for this purpose.
e) Local treatments for oral and genital ulcers.
f) Anti-TNF therapy: this new group of drugs is being evaluated in selected
g) Thalidomide is used to treat major oral ulcers in some centres.
treatment and follow-up of BS patients requires a team approach. Besides a
paediatric rheumatologist, an ophthalmologist, and a haematologist should be
included in the team. The family and patient should always be in touch with the
physician, or the centre responsible for treatment.
What are the side effects of drug therapy?
1) Diarrhea is the most common side-effect of colchicine. In rare cases, it may
cause a drop in the number of white blood cells or platelets. Azospermia (a
decrease in spermatozoon counts) has been mentioned, but this is not a major
problem in therapeutic doses.
2) Corticosteroids are the most effective anti-inflammatory drugs, but their use
is limited, because their long-term use is associated with serious side effects,
like diabetes mellitus, hypertension, osteoporosis, cataract formation and
retardation in growth. Children who have to be treated with steroids should
receive it once a day as a morning dose. For prolonged administration, calcium
preparations should be added to the treatment list.
3) Immunosuppressive drugs: Azathioprin may be toxic to the liver, may cause a
decrease in the number of blood cells and increase susceptibility to infections.
Cyclosporin-A is mainly toxic to kidneys, but can cause hypertension. It can
also cause an increase in body hair and problems with the gums. The side effects
of Cyclophosphamide are mainly depression of bone-marrow and bladder problems.
Long term administration interferes with the menstrual cycle and may cause
infertility. Patients under these treatments have to be followed closely and
should have blood and urine tests done every one or two months.
How long should treatment last for?
There is no standard answer to this question. Generally, the immunosuppressive
therapy is stopped after a minimum of two years, or when the patient has been in
remission for two years.
However, children with vascular and eye disease, where complete remission is not
easy to achieve, the therapy may be life-long. In such instances, the medication
and doses are modified according to clinical manifestations.
What about unconventional or complementary therapies?
There is no such therapy for BS.
What kind of periodic check-ups are necessary?
Periodic check-ups are necessary to monitor disease activity and treatment and
are especially important for children with eye inflammation. An eye specialist,
who is experienced in treating uveitis, should examine the eyes. The frequency
of check-ups depends on the activity of disease and the type of medication used.
How long will the disease last for?
Usually the course of the disease includes periods of remissions and
exacerbations. The overall activity, generally, decreases with time.
What is the long-term prognosis (predicted course and outcome) of the
There is no sufficient data on the long-term follow-up of patients with
childhood BS. From the data available, we know that there are many patients with
BS who do not need any treatment.
However, children with eye, nervous system, and vascular involvement require
special treatment and follow-up.
BS can be fatal in rare cases, mainly because of vascular involvement (rupture
of pulmonary arterial or other peripheral aneurysms), severe central nervous
system involvement and intestinal ulcerations and perforations, seen especially
among some ethnic groups of patients (Japanese).
The main cause of morbidity is eye disease, which can be very severe.
The child’s growth may be retarded, mainly secondary to steroid therapy.
Is it possible to recover completely?
Children with milder disease may recover, but the majority may have long periods
of remission, followed by flares of the disease.
How could the disease affect the child and family’s daily life?
Like any other chronic disease, BS does affects the child and the family’s daily
life. If the disease is mild, without eye and other major organ involvements,
usually the family may lead a normal life.
The most common problem with this group is the recurrent oral ulcers, which may
be troublesome in many children. These lesions may be painful and can interfere
with eating and drinking. Eye involvement may also be a serious problem for the
What about school?
It is essential to continue education in children with chronic diseases. In BS,
unless there is eye or other major organ involvement, the children with BS can
attend school regularly. Visual impairment may necessitate special educational
What about sports?
The child can attend sports activities if there is only skin and mucosa
involvement. During attacks of joint inflammation, sports should be avoided.
Arthritis in BS is short-lived and resolves completely. The patient may restart
sports after the inflammation is gone.
However children with eye and vascular problems have to limit their activities.
Prolonged standing should be discouraged in patients with vascular involvement
of the lower extremities.
What about diet?
There is no restriction with regard to food intake.
Can climate influence the course of the disease?
No there is no known effect of climate on the expression of BS.
Can the child be vaccinated?
The physician should decide about which vaccines the child can receive. If a
patient is being treated with an immunosuppressive drug (steroids, azathioprin,
cyclosporine-A, cyclophosphamide, anti-TNF etc.) vaccination with live,
attenuated viruses (such as anti-rubella, anti-measles, anti-parotitis,
anti-polio Sabin) have to be postponed.
Vaccines that do not contain living viruses, but only infectious proteins (anti
tetanus, anti diphtheria, anti polio Salk, anti hepatitis B, anti pertussis,
pneumococcus, haemophilus, meningococcus), can be performed.
What about sexual life, pregnancy, birth controls?
One of the major problems with sexual life is the development of genital ulcers.
They can be recurrent and painful and interfere with sexual intercourse. Females
with BS have mild disease, they will have a normal pregnancy.
Birth control should be considered if the patient is on immunosuppressive drugs.
Patients are advised to consult their physician about birth control and
الدكتور رضوان غزال MD, FAAP
- مصدر المعلومات : الجمعية الأوروبية لأمراض المفاصل عند الأطفال
last update 12.03.08
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