Behçet's Disease versus Behçet's Syndrome with Some Criteria for Systemic Lupus Erythematosus

Abstract

Behçet's disease is a relapsing multisystem inflammatory disease with the classic triad of orogenital ulcers and uveitis. Behçet's disease has certain diagnostic criteria, which although useful, are of little help in the early recognition of the disease or for the diagnosis of atypical, early, or incomplete forms. These criteria include the presence of recurrent oral ulcers plus two of the following: genital ulcerations, eye inflammation, skin lesions, and a positive pathergy test. Behçet's syndrome is a presentation resembling Behçet's disease but superimposed on an underlying disorder such as systemic lupus erythematosus (SLE), Crohn's disease, and mixed connective tissue disease. We report a patient with recurrent oral ulcers who subsequently developed genital ulcers 4 years later. She was also found to have a positive antinuclear antibody (ANA). The full diagnostic criteria for Behçet's disease, as a positive pathergy test, eye and skin changes were not present. She also did not meet the criteria for SLE as defined by the American Rheumatism Association. Thus, this patient may evolve into either Behçet's disease or SLE, or could have Behçet's syndrome with underlying SLE.