Disease: Behcet's Disease

Behcet’s disease is a rare autoimmune disease, and the exact cause remains unknown. What is known about this syndrome is that it results from damage done to the blood vessels. Because not much is known about this rare disease, scientists are exploring possible genetic, bacterial, and viral causes. It appears to be most common in Middle Eastern men; however most cases in the United States are women. Individuals with Behcet’s usually begin to show symptoms in their mid-20’s to 30’s, although symptoms can appear at any point in life. (8)

Symptoms that are the most common are mouth sores, genital sores, inflammation inside of the eye, and skin related problems. An over-active response to trauma in the skin is considered a symptom unique to Behcet's disease. At least half of those affected by Behcet’s have outbreaks of lesions, or sore in the mouth and/or genitals. They are painful and occur in clusters in the mucus membranes of these areas. Genital ulcers in females occur on the vulva and vagina, and in males they are found on the scrotum or penis. (8) Rashes or general irritation of the skin is also common in most cases.


In more than half of individuals affected by Behcet’s disease exhibit inflammation inside of the eye and this may also result in eye pain, blurred vision, and redness. Arthritis, blood clots, and inflammation of the central nervous and digestive systems are also related to the disease. (8) Symptoms can range from mild to severe and occur in an outbreak and remission fashion. (6)

When my sister was 15 years old, she was diagnosed with Celiac and Behcet’s. The inflammation caused by her Celiac disease most likely caused her Behcet’s. The first symptoms she showed were painful genital and oral sores, and she occasionally had outbreaks of these sores and rashes. In her case, her symptoms are mild and manageable. She is working toward having a completely gluten-free diet which will reduce the inflammation and in turn reduce her symptoms. She also had moderate asthma and diaper rashes as a child, which could also be linked to her Behcet’s. (9)

Depending on symptom severity, different treatments have been explored. Currently, corticosteroids, thalidomide, cyclosporine, colchicine, and tacrolimus are included in treatments of various cases. (6) Because this disease is rare and not much is known, research is limited. Behcet’s disease was discovered in 1937, when the Turkish dermatologist H. Behcet took extra notice in patients with both oral and genital sores. Certain symptoms can be specific to different ethnic groups, and these differences are being studied. (8)


References:

6. Mark R. Fleisher, James Fulmer, Kenneth Barwick, Kyle Etzkorn, Ruby Turner, Katherine Hopkins, A case report: Infliximab in the treatment of Bechet’s syndrome, The American Journal of Gastroenterology, Volume 96, Issue 9, Supplement 1, September 2001, Page S196
8. Resource center. American Behcet’s Disease Association http://www.behcets.com/site/pp.asp?c=bhJIJSOCJrH&b=1120453
9. Olivia Seirup, personal communication