Behcet Disease

Key Points
Rare disorder
Cause is unknown, thought to be an autoimmune disorder
Consists of ulcers in the mouth and on the genitals, lesions on the skin, and eye problems

Behcet disease is a rare autoimmune disorder that results in a chronic inflammation of blood vessels. It is characterized by years of various symptoms which recur, including sore throat/tonsillitis, joint/muscle pain, weakness, anorexia/weight loss, headache, and fluctuations in body temperature. However, the most common and earliest real sign of the disease is the formation of ulcers in the mouth, which occur in most patients. These ulcers can form anywhere in the mouth and last 1-2 weeks. Other symptoms include genital ulcers, uveitis (reddening and swelling of the eye tissue) which can lead to blindness if untreated, and painful skin lesions. Rarely, affected persons may experience gastrointestinal issues, joint swelling or pain, or problems with the nervous and circulatory system. Involvement of these systems leads to death in about 4% of cases.

Behcet disease is thought to be an autoimmune disorder, but the exact cause is unknown. It could be developed in conjunction with a bacterial or viral infection, or may be genetic, or a combination thereof. In any case, it is much more common in those of Mediterranean, Middle Eastern, and Far Eastern descent, occurring twice as much in these groups. In these groups it occurs more often in men, but with other groups, while the disease is even rarer, women are often affected more. It generally develops between 20 and 30 years of age, though it can develop at any age.

Differential Diagnosis (Other conditions with similar appearance)

Amyloidosis, AA (Inflammatory)
Systemic Lupus erythematosus
Antiphospholipid syndrome
Wegener granulomatosis
Inflammatory Bowel Disease
Paraneoplastic Syndromes
Polyarteritis nodosa

Diagnosis
Key Points
Diagnosis is difficult due to the infrequency and disparity of symptoms
Commonly diagnosed through the occurrence of a specific combination of symptoms within a specific time period
Pathergy test will be performed to confirm diagnosis

Behcet disease is difficult to diagnose, as symptoms are intermittent and do not always occur together. Diagnostic standard for the disease is a specific combination of the following symptoms during a twelve month period: 3+ episodes of recurrent Mouth ulcers in combination with any two other symptoms, including genital ulcers, uveitis, or skin lesions. A positive pathergy test, a skin prick test, can also confirm the existence of the disease.

Treatment
No cure for Behcet disease
Goal of treatment is to control symptoms to prevent complications
*Multiple doctors of different specialties may be required to treat the vast array of symptoms
OTC Options: Local oral anesthetics for Mouth ulcers

There is no cure for Behcet disease, so treatment consists of managing symptoms to prevent complications from those symptoms. Often, health care professionals of varying specialties are needed to combat the vast array of symptoms. With time, affected persons may experience longer and longer time periods between flare-ups, eventually entering remission, though the disease can reappear at any time.

Topical treatments such as tetracycline solution, topical corticosteroids, and local anesthetics are used to treat ulcers and lesions. For treatment of systemic symptoms, health care professionals may prescribe oral corticosteroids, anti-inflammatories, or immunosuppressants. There are several drugs being used in experimental treatments for the disease, including colchicines, Cyclophosphamide, Thalidomide, Infliximab and potassium iodide.