Differential Diagnosis

Multiple diseases can present with findings similar to those seen with Adamantiades-Behget's disease and should be considered when a patient presents with recurrent oral or genital ulcers, inflammatory eye disease, or other manifestations of vasculitis. Included in the differential diagnosis are systemic lupus erythematosus (Chapter 1), seronegative spondyloarthropathies, inflammatory bowel disease (Crohn's or ulcerative colitis) (Chapter 20), herpes or other viral infections (Chapter 10), other forms of vasculitis (Chapter 8), and inflammatory skin diseases such as pemphigus vulgaris or pemphigoid lesions (Chapter 37). All patients presenting with oral and genital ulcerations should undergo testing for herpes simplex virus using culture or polymerase chain reaction methods, to ensure that viral infection is not present.

TABLE 2 International Study Group Criteria for the Diagnosis of Behget's Disease3

Recurrent oral ulceration

Minor aphthous, major aphthous, or herpetiform ulceration observed by a physician or patient that recurred at least three times in one 12-mo period Plus, two of the following criteria Recurrent genital ulceration

Aphthous ulceration or scarring observed by physician or patient Eye lesions

Anterior uveitis, posterior uveitis, or cells in vitreous on slit-lamp examination Or

Retinal vasculitis observed by ophthalmologist Skin lesions

Erythema nodosum observed by a physician or patient, pseudofolliculitis, or papulopustular lesions

Acneiform nodules observed by a physician in postadolescent patients not on corticosteroid treatment Positive result on pathergy testingb Read by a physician at 24-48 hr aFindings applicable only in the absence of other clinical explanations (systemic lupus erythematosus, inflammatory bowel disease, seronegative spondyloarthropathies, and viral infections).

bPathergy testing is performed by pricking the skin with a 20-25 gauge needle. A positive test is defined as a papular/ pustular lesion >2 mm that appears at the site 24-48 hr later. Source: From Ref. 30.

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