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Behcet's disease cannot be confirmed through clinical laboratory results. Usually you will be assigned to an immunologist or rheumatologist. Diagnosis of Behcet disease is based on clinical criteria because of the absence of a pathognomonic laboratory test. The period between the appearance of an initial symptom and a major or minor second manifestation can be up to a decade long in many cases. The number of different criteria/classification systems that have been introduced over the past 25 years reflects the failure of any single one to meet clinical demands.

The revised 1987 criteria of the Japanese group (Shimizu) have been widely applied (Table 1 below). More recently, the diagnostic criteria of the International Study Group for Behcet Disease (ISGBD) have been applied in establishing firmer diagnoses (Table 2 below). The major limitation of these criteria, however, lies in the fact that recurrent oral ulceration is the linchpin for diagnosis of Behçet disease. For example, patients with uveitis and genital ulcer, without oral aphthosis, would not be considered to have Behçet disease, although this is in fact a far-advanced form of the disease. Therefore, we recommend that the Japanese criteria be applied concurrently with the ISGBD criteria until a more exact system is devised. Behcet's can be called either a disease or syndrome ( combination of symptoms indicating a particular disease).
Behcet's can also be classified under different types of 'Behcet's Diagnosis'. Such as Vasculo-Behcet's, Neuro-Behcet's, Occulo-Behcet's and so on so forth.

The symptoms do not have to occur simultaneously, but you must have had mouth ulcers with at least two other symptoms. The two other symptoms can occur on separate occasions This is why Behcet's is difficult to diagnose.
There are other symptoms associated with Behcet's, which are gone into detail on the 'symptoms page'. These are arthritis, gastro-intestinal problems, superficial thrombo-phlebitis, deep vein thrombosis, hearing and balance problems, central nervous damage mimicking MS ( multiple sclerosis ), benign intracranial hypertension and meningo-encephlitis. Don't panic though, as in the majority, Behcet's tends to take a mild form. It is difficult for your physician to make a diagnosis, and it may be the case that you are diagnosed under the banner of 'suspected Behcet's' if your symptoms are not clearly defined under the international diagnostic criteria.

If you do have the classic symptoms you will be given a definite diagnosis. This is why we stress that if you have 'suspected Behcet's, you monitor and document 'ANY' symptoms that you may experience, however minor these may seem to you.

It is usually the case that to have a confirmed diagnosis of Behcet's, you should have recurrent mouth ulcers. However about 5% of known cases of Behcet's patients do not have mouth ulcers. It could be possible that you experience nasal or throat ulcers, these could come under the same category.

If you read below, you will see there are other symptoms associated with Behcet's, but you may not necessarily experience them all.

How is Behcet's Syndrome diagnosed?

Table 1. Diagnostic criteria of the Behcet syndrome research committee of Japan 1987 revision Major Recurrent aphthous ulceration of the oral mucous membrane

Major

Recurrent aphthous ulceration of the oral mucous membrane

Skin Lesions

Erythema nodosum
Subcutaneous thrombophlebitis
Folliculitis, acnelike lesions
Cutaneous hypersensitivity

Eye lesions

Iridocyclitis
Chorioretinitis, retino-uveitis
Definite history of chorioretinitis or retino-uveitis

Genital ulcers

Minor

Arthritis without deformity and ankylosis

Gastrointestinal lesions characterised by ileocecal ulcers

Epididymitis

Vascular lesions

Central nervous system symptoms

Diagnosis

Complete

Incomplete





Suspected



4 major features

3 major features
or 2 major + 2 minor
or typical ocular symptom + 1 major or 2
minor features

2 major features
or 1 major + 2 minor

·         Table 2. International criteria for classification of Behcet disease

1990

Recurrent oral ulceration

Minor aphthous

Major aphthous or herpetiform ulceration observed by a physician

or reported reliably by patient

Recurrent at least 3 times in one 12-month period

Plus 2 of the following:

Recurrent genital ulceration

Recurrent genital aphthous ulceration or scarring, especially males, observed by a physician or reliably reported by patient

Eye lesions

Anterior uveitis
Posterior uveitis
Cells in vitreous on slit lamp examination

or

Retinal vasculitis observed by
physician (ophthalmologist)

Skin lesions

Erythema nodosum-like lesions observed by physician or reliably reported by patient

Pseudofolliculitis
Papulopustular lesions

or

Acneiform nodules consistent with Behcet disease observed by a physician and in postadolescent patients not receiving corticosteroids

Positive pathergy test

An erythematous papule, > 2 mm, at the prick site 48 h after the application of sterile needle, 20-22 gauge, which obliquely penetrated avascular skin to a depth of 5 mm:read by physician at 48 h

What causes the symptoms and disturbances of Behcet's Syndrome?

The exact cause of Behçet’s disease is unknown. Most of the symptoms are caused by inflammation of the blood vessels, particularly veins. Inflammation is the body’s characteristic reaction to injury or disease and is marked by four signs: swelling, redness, heat, and pain.

The problem with Behcet's is the unpredictability of when and how major the outbreak will be. Doctors think that an autoimmune reaction may cause blood vessels to become inflamed, but they do not know what triggers this reaction.

We all have our own theory's, and one that is subscribed to is having a dormant 'Behcet's gene. This dormant Behcet gene is awoken by a trauma or even a series of trauma's. The trauma can range from having a series of viral infections, which accumulated can trigger the gene to having been subjected to high stress levels, which again, in the right circumstance, can trigger the Behcet gene. It may also have a connection with the bodies chemical imbalance during these episodes.

In an autoimmune reaction, the immune system mistakenly attacks and harms the body’s own tissues. 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. Indeed, treatments are now concentrating on introducing a 'Trojan' germ. What this 'Trojan' germ will do, is tell the bodies defence system to attack 'it' instead of the bodies tissues. Very shortly we will be putting 'exclusive' details of one of these such treatments.
( You could assimilate the inflammation to say "arthritis" in your veins instead of in your joints).

Behçet’s disease is not contagious and does not spread from one person to another. Researchers think that two factors are probably important in its development. First, it is believed that abnormalities of the immune system make some people susceptible to the disease. Researchers think that this problem may be inherited; that is, it may be due to one or more specific genes. Second, something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people. Researchers have found that people who have frequent strep infections (caused by Streptococcus bacteria) are more likely to develop Behçet’s disease.

We believe that each of us has a trigger. A trigger could be stress, dietary or even externally influenced. Flair up's can follow a stressful event and that event could be anything. Sulphur is another.

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