DESTIN, FLA. – Although no agent has been approved for the treatment of Behçet's disease in the United States, several treatments have been tried in patients with mucocutaneous manifestations of the disease, said Dr. Kenneth Calamia.
Behçet's disease is relatively rare in the United States and Europe, occurring in 0.3 to 7.5 per 100,000 people, but among those affected, mucocutaneous symptoms are common, and in fact are among the hallmarks of the disease. In a study of 164 patients treated from 1985 to 1997 at the Mayo Clinic in Jacksonville, Fla., where Dr. Calamia is associate professor of medicine, 98% had oral ulcers and 80% had genital ulcers. These were the most common manifestations, he said.
According to 2008 EULAR guidelines for the treatment of Behçet's, the decision to treat skin and mucosal involvement should be based on the severity perceived by the physician and patient, and mucocutaneous involvement should be treated according to the dominant or codominant lesions present (Ann. Rheum. Dis. 2008;67:1656-62).
Topical treatments should be used first line for isolated oral and genital ulcers and acnelike lesions; colchicine should be used when the dominant lesion is erythema nodosum; and azathioprine, interferon-alpha, and tumor necrosis factor (TNF)-alpha antagonists can be considered in resistant cases, according to the guidelines.
The list of treatments used for the management of mucocutaneous manifestations includes these and other agents. One of Dr. Calamia's favorite concoctions for oral ulcers is “magic mouthwash,” an elixir of half Decadron (betamethasone) syrup or Celestone syrup and half Benadryl topical anesthetic. “This can control ulcerations very well, especially if used early at the first sign of a breakout,” he said. The elixir is used before meals and at bedtime, and is swished in the mouth, held as long as possible, and spit out.
According to Dr. Calamia, other treatments that have been used for mucocutaneous manifestations of Behçet's include topical steroids, which work and have a better side effect profile than do systemic treatments; topical tacrolimus, pimecrolimus, and pentoxifylline, which dermatologists particularly like; azathioprine, which works; interferon-alpha, which also works; thalidomide, which works but causes neuropathy, so it is no longer used for this disease; dapsone, which is another favorite of dermatologists; methotrexate, which is used but which he is “not personally impressed with” for mouth ulcers; colchicine, which can be used for this or any of the other manifestations of the disease; and anti-TNF agents, which are clearly of benefit.
In a randomized, placebo-controlled study of etanercept, 40% of 40 patients who received etanercept were ulcer free at 4 weeks, compared with just 5% of those who received placebo. There was a significant reduction in oral ulcers, modular lesions, skin lesions, and arthritis attacks at 4 weeks, he said (J. Rheum. 2005;32:98-105).
“There's no question that anti-TNF drugs do work, but it is probably best to try nonbiologics in these patients, especially for mucocutaneous disease,” said Dr. Calamia, who disclosed that he has received research support from Genentech and Celgene and served on an advisory board for Centocor.