LAKE BUENA VISTA, FLA. — The diagnosis of Behçet's disease must be considered in any patient with recurrent oral and vulvar aphthous ulcers, even if the deep, full-thickness ulcers in the mouth and vulva develop at different times.
Behçet's disease is a chronic inflammatory vasculitis most commonly seen along the ancient silk route from Japan and across Korea, Turkey, and Greece, according to Dr. Andrew T. Goldstein. In the West, it occurs most often among young women of Asian or Mediterranean descent.
“This is a bad vasculitis, with complications including dissection of the aorta, blindness, and stroke,” he said.
Aside from the aphthous ulcers, patients with Behçet's disease may have acnelike skin lesions or erythema nodosum as well as ocular, central nervous system, and bowel involvement. The ocular manifestations can be varied and severe, and include iritis, uveitis, and retinal vasculitis. Behçet's disease also can be associated with arthritis and meningitis, and any evidence of this disorder should prompt consultations with ophthalmologists, rheumatologists, and gastroenterologists as symptoms dictate.
“One of the easiest ways of diagnosing Behçet's is the pathergy test,” said Dr. Goldstein, who practices in Washington.
The pathergy test, in which a 5- to 7-gauge needle is inserted into the forearm, has a very high predictive value, although its negative predictive value is lower. If induration develops 24–48 hours later at the site of needle insertion, the test is positive, he said at the annual meeting of the International Pelvic Pain Society.
Although a positive pathergy test is helpful in the diagnosis of Behçet's disease, only a minority of Behçet's patients demonstrate the pathergy phenomenon, according to the Vasculitis Foundation. Patients from the Mediterranean region are more likely to show a positive response, with only 50% of patients in Middle Eastern countries and Japan showing the reaction. A positive reaction is even less common in the United States, and other conditions can occasionally mimic the results (www.vasculitisfoundation.org/pathergytest
Treatments that have been tried for Behçet's disease include conventional immunosuppressives such as azathioprine and corticosteroids; and anti-tumor necrosis factor therapy, particularly with infliximab, according to Dr. Goldstein, who also practices at George Washington University Hospital, Washington.
A recent international expert panel suggested that anti-TNF therapy might be suitable for patients with severe, organ-threatening disease—patients with two or more relapses of posterior uveitis per year—low visual acuity resulting from chronic cystoid macular edema, or active central nervous system disease (Rheumatology 2007;46:736–41).
Aphthous ulcers not caused by Behçet's disease occasionally can be associated with Epstein-Barr virus or cytomegalovirus, but most commonly they are a manifestation of stress.
“These can be seen in adolescents who are sexually naive and are accused of having herpes and are repeatedly tested and cultured,” Dr. Goldstein said.
Treatment for these ulcers involves managing pain with lidocaine and systemic pain medications, intralesional triamcinolone, and amoxicillin-clavulanate and fluconazole for superinfection.
Pain due to vulvar lesions in Behçet's disease may respond to lidocaine. Courtesy Dr. Andrew T. Goldstein