August 2016 | Case of the Month

The diagnosis is Behçet syndrome.

Behçet syndrome is a rare condition characterised by the association of recurrent aphthous stomatitis with genital ulceration and eye disease, ulcers are painful and tend to scar. There may be a number of other systemic, especially neurological, or cutaneous manifestations. Behçet syndrome usually starts in the third and fourth decade, the cause is unknown. The international criteria for classification of Behçet syndrome defines the condition as at least 3 episodes of recurrent oral ulcers in a 12-month period plus at least two more of the following:

• Genital ulcers
• Eye involvement - uveitis or retinal damage
• Skin lesions - erythema nodosum, papulopustular lesions and acneform nodules
• Pathergy - the pathergy cutaneous test is a pricking of the skin made by the doctor with a sterile needle. The results are read after 24-48 hours. In patients with Behçet syndrome the test is often positive with formation of a pustule at the test site

Unlike recurrent aphthous stomatitis, Behçet syndrome is not self‐limiting. It causes morbidity (especially in terms of ocular and neurological disease) and occasional mortality. Most patients present with oral and ocular disease but there follows a relapsing and remitting but variable course. CNS involvement, thromboses of major vessels and gastrointestinal perforation result in a poor prognosis
Patients with suspected Behçet syndrome require prompt referral to a specialist.

For more information on oral lesions please click on the following link - oral lesions.