Saturday, March 15, 2008

Behçet Disease is Not a Melungeon Disease

by Janet Crain

As promised, I have researched Behcet Disease to report on whether or not it is a Melungeon Disease as many websites state.

Happily, I can report that Behcet Disease is NOT a Melungeon Disease.

Behcet's is not hereditary, so it cannot possibly be caused by your genetic background.

And the fact it is considered more prevalent in the areas surrounding the old silk trading routes has no bearing, in any case, because there is no link to this area for Melungeon descendants.

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No one knows why the immune system starts to behave this way in Behçet disease. It is not because of any known infections, it is not hereditary, it does not have to do with ethnic origin, gender, life-style, or age, where someone has lived or where they have been on holiday. It is not associated with cancer, and links with tissue-types (which are under investigation) are not certain. It does not follow the usual pattern for autoimmune diseases.

http://en.wikipedia.org/wiki/Behcet_disease#Causes

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Epidemiology

Behçet disease is considered more prevalent in the areas surrounding the old silk trading routes in the Middle East and in Central Asia. Thus, it is sometimes known as Silk Road Disease. However, this disease is not restricted to people from these regions.

An estimated 15,000 to 20,000 Americans have been diagnosed with this disease. In the UK, it is estimated to have about 2 cases for every 100,000 people.

Globally, males are affected more frequently than females. In the United States, more females are affected than males.

Clinical manifestations

Behcet disease is a chronic relapsing multisystem disorder with complete or incomplete remission between attacks. The diagnosis is based on clinical findings. Various sets of diagnostic criteria have been published, with general agreement on the importance of mucocutaneous and ocular manifestations. Because symptoms appear asynchronously, the criteria may not be met in many patients (75%) at the time of observation. According to the work of the International Study Group, the major diagnostic criteria for Behcet disease are:

(1) oral ulcers recurring at least 3 times per year
(2) genital ulcers or scars
(3) eye involvement
(4) skin lesions (erythema nodosum, folliculitis, acneiform lesions)
(5) pathergy skin test observed by a physician

Minor diagnostic criteria include arthritis or arthralgia, deep venous thromboses, subcutaneous thrombophlebitis, epididymitis, family history, and gastrointestinal, CNS, or vascular involvement.

Oral ulcers plus 2 other major criteria are required for the diagnosis (International Study Group for Behcet's disease 1990). The terms "complete" and "incomplete" Behcet disease are based on the number of major criteria observed. The requirement for oral ulcers excludes the 3% of all patients who do not have this symptom. Considerable differences exist between the diagnostic criteria of 3 groups: among patients diagnosed according to Mason and Barnes (Mason and Barnes 1969), 17% do not meet the criteria of the International Study group and 46% do not meet those of the Japanese group (Feutrie et al 1994). Therefore, these criteria should be used to establish standards for homogenous patient populations in studies, research, or teaching rather than to diagnose individual cases. A revision of criteria is planned in the near future.

http://www.medlink.com/medlinkcontent.asp

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by Marjan Yousefi, MD, Department of Dermatology, Geisinger Medical Center

Background

Behçet disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis.

This complex, multisystemic disease includes involvement of the mucocutaneous, ocular, cardiovascular, renal, gastrointestinal, pulmonary, urologic, and central nervous systems and the joints, blood vessels, and lungs.

It is characterized by oral aphthae and by at least 2 of the following: (1) genital aphthae, (2) synovitis, (3) posterior uveitis, (4) cutaneous pustular vasculitis, (5) meningoencephalitis, (6) recurrent genital ulcers, and (7) uveitis in the absence of inflammatory bowel disease or collagen vascular disease.

http://www.emedicine.com/DERM/topic49.htm

Diagnosis

There is no specific pathological test for Behçet disease at present. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks. Other causes for these symptoms have to be ruled out before making the diagnosis. The symptoms do not have to occur together, but can have happened at any time.

No one knows why the immune system starts to behave this way in Behçet disease. It is not because of any known infections, it is not hereditary, it does not have to do with ethnic origin, gender, life-style, or age, where someone has lived or where they have been on holiday. It is not associated with cancer, and links with tissue-types (which are under investigation) are not certain. It does not follow the usual pattern for autoimmune diseases.

http://en.wikipedia.org/wiki/Behcet_disease#Causes

Disclaimer:

This article is not intended to provide medical advice or diagnosis. Consult a medical health professional if you think you might be suffering from a medical condition.

1 comment:

Anonymous said...

More understanding is needed on your part...not about Behcets but genetics. Behcets does have an absolute component; hence the allele testing. if the alleles were not important then you could say there was no genetic influence . but, since alleles on GENES must be "turned on" so to speak...then Behcets is a genetic disorder that must be "triggered" by environmental factors....these factors are things like very particular viruses or bacteria. Examples of these are Strep, Mono, Chicken pox, Mumps....all the types of infections that the immune system remembers for LIFE . These may at anytime trigger the ALLELE on the GENE causing the immune system to malfunction. HLA- B51 is the most prominent gene associated with Behcets but not everyone has the B51 gene. however, those who develop Behcets have B51 gene...that's why it's part of the work up.

Dr John S Purifoy III