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Behcet's Disease

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About Behcet's Disease

Behcet's disease, also called Silk Road Disease, is a poorly understood condition. But is thought to be an autoimmune disease of the blood vessel walls.

Behcet's Disease and IBD

Many Gastrointestinal (GI) Symptoms and Extraintestinal Manifestations of Behcet's Disease similar to those seen in Inflammatory Bowel Disease (IBD) and Celiac Disease. This can make a Differential Diagnosis difficult.[8]
Diagnosis of Behcet's Disease
There are no clear Diagnotic tests for Behcet's Disease and diagnosis relies on symptomatic presentation.
Symptoms of Behcet's Disease
Behçet's Disease involves the following Symptoms which cannot be explained by another disease, condition, or injury.

- Recurrent Oral (Mouth) Aphthous Ulcers or Sores
--- Aphthous Ulcers, or Oral Ulcerations, that occur 3x or more in the period of a year is the hallmark symptom for Behcet's diagnosis.

PLUS at least 2 of the Following Symptoms:
- Genital Sores / Ulcerations (Not attributed to other disease such as Herpes virus infection, etc.)

- Eye Symptoms
--- Uveitis
--- Presence of Cells in Vitreous Region of the Eye
--- Vasculitis of the Retina

- Skin Lesions (*patient may not be on steroid therapy when this symptom is observed)
--- Erythema Nodosum
--- Pseudofolliculitis
--- Papulopustular Lesion
--- Acne - like skin lesions

- Positive Pathergy Test
Epidemeology of Behçet's Disease
- Although Behcet's Disease can occur in anyone, it is most prevalent in persons of Asian or Middle Eastern descent. Recent studies have shown that Behcet's Disease in the United States may be more prevalent than previously thought [2]

- Behcet's Disease age of onset is in adulthood between approximately 25 and 30 years of age. [2]
Genetics of Behcet's Disease
The specific factors that cause Behcet's Disease are not fully understood but the following genes have a positive association with the disease:
- HLA-B*51
- IL10 - Interleukin 10
- IL23R-IL12RB2

References

1. Tamer İrfan Kaya, “Genetics of Behçet's Disease,” Pathology Research International, vol. 2012, Article ID 912589, 6 pages, 2012. doi:10.1155/2012/912589 http://downloads.hindawi.com/journal...012/912589.pdf

2. Calamia K. 2001. Hoffman/Weyand: Inflammatory Disease of Blood Vessels Marcel Dekker, Inc., NY. American Behcet's Disease Association, ABDA. Accessed June 2012. http://www.behcets.com

3. http://www.nature.com/nmiddleeast/20....2010.183.html

4. Remmers E, Cosan F, Kirino Y, et al. Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet's disease. Nature Genetics. 2010; 42: 698-702.

5. http://emedicine.medscape.com/article/329099-clinical

6. http://www.patient.co.uk/doctor/Behcet's-Disease.htm

7. Din E, Mat C, Hamuryudan V, et al. Papulopustular skin lesions are seen more frequently in patients with Behçet's syndrome who have arthritis: a controlled and masked study.Ann Rheum Dis. 2001;60:1074-1076. http://ard.bmj.com/content/60/11/1074.full.pdf+html

8. Aysin Kokturk, “Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease,” Pathology Research International, vol. 2012, Article ID 690390, 9 pages, 2012. doi:10.1155/2012/690390 http://downloads.hindawi.com/journal...012/690390.pdf

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