Behcet's Disease

Behcet's disease is a multisystem disease marked by exacerbations and remissions. Symptoms include recurrent oral and genital ulcerations, a variety of ocular manifestations, and cutaneous lesions. The first symptom is frequently aphthous stomatitis, which may precede other disease manifestations by months or years. The oral ulcers are painful and appear as circular erythematous lesions with a yellow pseudomembrane. Genital lesions involve the vulva in women and the scrotum and penis in men. They are painful, often larger than the oral lesions, and have irregular margins. Cutaneous manifestations are varied and include sterile vesiculopustules in an acral or facial distribution, pustules, purpura (secondary to vasculitis), erythema nodosum and pyoderma gangrenosum. Superficial thombophlebitis also occurs. Pathergy, the occurrence of lesions after trivial trauma, is often present. Ocular involvement is the most significant complication of Behcet's disease, occurring in 90% of cases. Although posterior uveitis is the most frequently observed manifestation, a wide variety of lesions are possible. A variety of other organ systems can be involved such as: non-erosive mono- and polyarthritis; gastrointestinal pain, bleeding, or even perforation; neurologic involvement, including meningoencephalitis, cranial nerve palsies and pyramidal and extrapyramidal symptoms; vascular aneurysms, occlusive vascular disease, and coronary artery arteritis; and myocarditis or valvular disease.

Behcet's disease is most likely an autoimmune disease with a genetic disposition. The lesions result from circulating immune complexes and a neutrophilic reaction (i.e. infiltration of neutrophils around blood vessels). The disease occurs primarily in Japan, the Middle East, and the Mediterranean region.

Behcet's disease is diagnosed based upon criteria designated by an International Study Group (see below), though a number of other diagnostic criteria have been used in the past.

International Study Group Criteria for Diagnoses of Behcet's Disease

Recurrent oral ulceration occurring at least three times in a 12 month period

and at least two of the following

Genital ulceration: Recurrent genital ulcerations

Eye Involvement: Anterior or posterior uveitis; retinal vasculitis; cells observed in vitreous on slit-lamp

Cutaneous lesions: Erythema nodusum; papulopustules; pseudofolliculitis; acneiform nodules (post-puberty and in absence of steroid use)

Pathergy (occurrence of lesions after trivial trauma)

Treatment is directed at the specific lesion and the involvement of vital organs, such as the eye. Early treatment is imperative to prevent systemic complications. Isolated mucocutaneous lesions can be treated symptomatically with viscous lidocaine or topical or intralesional steroids. Oral colchicine and dapsone have also been reported to be effective. More severe mucocutaneous disease may respond to thalidomide, methotrexate or oral steroids. For systemic involvement, immunosuppressive regimens are usually advocated, including prednisone, azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, IVIG, and TNF-α blockers such as entanercept or infliximab.

Oral ulcers heal in about 10 days. However, the disease is chronic and lesions tend to recur. Ocular involvement can lead to blindness.