Behcet's Disease
Behcet's disease is a rare,
chronic, lifelong disorder that involves inflammation of blood vessels throughout the
body. Symptoms of Behcet's disease include recurrent oral ulcers (resembling canker
sores), recurrent genital ulcers, and eye inflammation. The disorder may also cause
various types of skin lesions, arthritis, bowel inflammation, and meningitis (inflammation
of the membranes of the brain and spinal cord). Behcet's disease generally begins when
patients are in their 20s or 30s, although all age groups may be affected. Behcet's is a
multisystem disease; it may involve all organs and affect the central nervous system,
causing memory loss and impaired speech, balance, and movement. The effects of the disease
may include blindness, stroke, swelling of the spinal cord, and intestinal complications.
Is there
any treatment for Behcet's Disease
Treatment for Behcet's disease is symptomatic and supportive. Medication may be prescribed
to reduce inflammation and/or regulate the immune system.
What is the prognosis?
Behcet's disease is a lifelong disorder which proceeds over a long period of time in a
series of remissions (lack of disease activity) and exacerbations (periods of active
disease).
Diagnosis of Behcet's Disease
There is
no specific "Behcet’s test". Consequently, the diagnosis is based on the
clinical signs and symptoms that are compatible with the disease - such as the presence of
oral/genital ulcers, uveitis and skin lesions, etc.
An international group of physicians has established a set of guidelines to aid in the
classification of Behcet’s patients for the purpose of conducting research in the
disease. The criteria put forth by the group include recurrent oral ulceration (at least
three occasions in a year). In addition, a patient must also meet two of the following
four criteria for Behcet’s disease: recurrent genital ulcerations, eye lesions
(uveitis or retinal vasculitis), skin lesions (erythema nodosum, lesions, acne), and or
positive "pathergy test".
The pathergy test is a simple test in which the forearm is pricked with a small, sterile
needle. Occurrence of a small red bump or pustule at the site of needle insertion
constitutes a positive test. Although a positive pathergy test is helpful in the diagnosis
of Behcet’s, only a minority of Behcet’s patients demonstrates the pathergy
phenomenon (i.e., have positive tests). Patients from the Mediterranean region are more
likely to demonstrate pathergy. In addition, other conditions can occasionally result in
positive pathergy tests, so the test is not 100% specific |
