DEAR DR. GOTT: My sister is a 50-year-old widow with four children who has been diagnosed with Behcet’s disease. Her symptoms began last spring with some arthritis. She then developed horrible sores on her body and a terrible one in her throat. Then her eyes became involved. She has had to quit work and is in almost constant pain. Some nights she has to go to the hospital because she cannot stand the pain.
She has seen a number of specialists, but this is tiring for her and usually involves travel. We live in a small Canadian province without much diversity, so it was only by chance that the ophthalmologist she saw recognized the symptoms because he was from another country.
She has been getting injections in her eye and having both eyes checked frequently. Her throat is getting worse. Should she stop eating and drinking and have IVs until her throat heals? When she does see a physician she hasn’t seen before, they question whether she has lupus, Lyme or Crohn’s disease. She’s very discouraged.
Could you tell us what you think the treatment should be and if there are any centers in Canada or the United States that have expertise dealing with this?
DEAR READER: I must agree that your sister was extremely lucky to have had an ophthalmologist who recognized her symptoms and could put a name to her condition. My guess is he may be Asian, since Behcet’s is most common in the Far East and Mediterranean areas. The disease is rare in the United States and Canada, but cases do surface. It is named after a Turkish dermatologist, Hulusi Behcet, who documented aphthous oral ulcers, recurrent eye inflammation and genital lesions in the 1930s. Epidemiology of the disease is not well understood.
The syndrome is an idiopathic disorder classified as a form of vasculitis (blood-vessel inflammation). Because of the diversity of the blood vessels affected, it can occur throughout the body but is most common in the eyes, mouth, lungs, joints, genitals, brain, GI tract and on the skin. When the eyes are affected, inflammation can appear in the front or back, or sometimes in both areas at the same time. When the mouth, lips, tongue and inside of the cheeks are affected, the lesions are often painful, large and numerous. Aneurysms in the lungs can rupture and lead to massive hemorrhaging. Painful ulcers can develop on the vulva or scrotum. Involvement of the central nervous system is a dangerous manifestation. When the disease involves the white matter of the brain and brainstem, stroke, headache, confusion and other conditions can develop. The GI tract can be involved from the mouth to the anus. Inflammatory bowel disorders such as Crohn’s disease share similar side effects that may be difficult to differentiate from Behcet’s.
Diagnosis of Behcet’s can be extremely difficult to clarify. More than anything, symptoms such as oral or genital ulcers and evidence of vasculitis through biopsy of an involved organ are what lead a physician to make the call. Diagnostic guidelines involve two of the following: eye lesions and/or skin lesions a minimum of three times a year; recurrent genital ulcerations; and/or pathergy testing, which is a simple needle prick on the arm. A small red pustule occurring at the site of the needle insertion within two days is classified as positive. Pathergy is not proof positive of the disease, because many people from the Mediterranean will test positive and may not have Behcet’s.
For lesions confined to the mouth, genitals and skin, topical steroids and some oral drugs may be effective. When symptoms become particularly troublesome, corticosteroids may be beneficial. Some people may be placed on low-dose prednisone for control. When eye or central-nervous-system involvement occurs, high doses of prednisone and another form of immunosuppressive treatment are often necessary for control. Thalidomide, a drug banned in the United States because of a connection with birth defects, has been found to be effective in severe mucocutaneous cases of Behcet’s, even though there is a connection with the development of peripheral neuropathy in patients who take it for extended periods of time.
I do not believe that fasting is an answer for your sister, even with her troublesome throat lesion. In terms of finding a physician to provide care, while the condition is uncommon in the United States and Canada, it has been studied and documented extensively. In fact, a 1998 article by David Hellmann, published and copyrighted by the American College of Physicians in the Medical Knowledge Self-Assessment Program, edition 11, Rheumatology Section, covered Behcet’s. Furthermore, your sister could check with the Johns Hopkins Vasculitis Center to determine whether any trials she can participate in are under way. Good luck.
About Dr. Gott