Tom is a 30-year old who emigrated from Turkey three years ago.  He has had recurrent painful oral and genital ulcers for 10 years.  He has also experienced intermittent redness and pain of the eyes associated with blurred vision and sensitivity to light.

Behçet’s disease is a rare, chronic, autoimmune disorder characterized by inflammation of the arteries or veins (vasculitis).  The disease is named for the Turkish dermatologist Dr. Hulusi Behçet who, in 1937, described a triad of symptoms including oral ulcers, genital ulcers, and recurrent eye inflammation in some patients.  The precise cause of Behçet’s disease is unknown.  Vasculitis causes inflammation of the vessels and is the result of an exaggerated immune response to undefined environmental triggers such as infection or chemicals, particularly in genetically predisposed individuals.

Behçet’s disease is most common along the Old Silk Road, which spans the region from Japan and China in the Far East to the Mediterranean Sea, including countries such as Turkey and Iran.  The disease is most prevalent in Turkey, occurring in approximately 400 cases of every 100,000 individuals.  Behçet’s disease tends to develop in young adults, typically in their twenties and thirties.  In Middle Eastern countries, males are more commonly affected.  In the United States, other Western countries, Japan, and Korea the disease occurs more frequently in women.  Symptoms vary from person to person depending on the severity of blood vessels and organs involved.  If a blood vessel becomes inflamed and narrowed, the affected tissues or organs become ischemic and can even lead to death.

The most common symptoms of Behçet’s include oral ulcers, genital ulcers, and inflammation of the eyes, skin rash, and arthritis.  Patients frequently present painful oral ulcers, genital ulcers of the scrotum or vulva.  Pustular skin lesions (folliculitis) or tender nodules (erythema nodosum) can also occur.  Patients can experience pain, blurred vision, light sensitivity, or redness of the eyes.  Additional symptoms may also include blood clots and inflammation of the digestive tract or brain.

Treatment of Bechet’s disease is aimed at reducing symptoms and preventing complications.  Prognosis varies based on the disease severity and the organ system involved.  Topical corticosteroids are applied directly to the ulcers and skin lesions to relieve pain and discomfort.  Colchicine or Dapson can be used on mild ulcers and skin lesions.  Oral corticosteroids are particularly effective in reducing moderate to severe inflammation of mucosal ulcers and organ involvement.  Other treatments such as Methotrexate, Azathiporine and Cyclosporin, may be used for moderate to severe inflammation and to decrease the long-term side effects of high dose steroids.  Anti-TNF biologics such as Remicade (infliximab) have proven to be effective in treatment of severe Behcet’s disease.

Even though severe Behçet’s disease is a debilitating illness, early diagnosis and proper treatment is crucial to the prevention and treatment of complications.

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