Behçet syndrome, also known as Behçet disease, is characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis.
| Finding |
Definition |
| Recurrent oral ulceration | Minor aphthous, major aphthous, or herpetiform ulcers observed by the physician or patient, which have recurred at least three times over a 12-month period |
| Recurrent genital ulceration | Aphthous ulceration or scarring observed by the physician or patient |
| Eye lesions | Anterior uveitis, posterior uveitis, or cells in the vitreous on slit-lamp examination; or retinal vasculitis detected by an ophthalmologist |
| Skin lesions | Erythema nodosum observed by the physician or patient, pseudofolliculitis, or papulopustular lesions; or acneiform nodules observed by the physician in a postadolescent patient who is not receiving corticosteroids |
| Positive pathergy test | Test interpreted as positive by the physician at 24 to 48 hours |
For the diagnosis to be made, a patient must have recurrent oral ulceration plus at least two of the other findings in the absence of other clinical explanations.
References:
- O’Neill TW, Rigby AS, Silman AJ, Barnes C. Validation of the International Study Group criteria for Behçet’s disease. Br J Rheumatol. 1994 Feb;33(2):115-7. [Medline]
Created: Sep 10, 2007
