Behcet’s disease is a heterogenous condition of unknown etiology with variable involvement of several organ systems. An international group of experts evaluated patients from 7 countries in an attempt to develop a diagnostic criteria that would be simple yet have improved discriminatory performance compared to previous diagnostic systems.
Diagnostic features - both of the following:
(1) Recurrent oral ulcerations, having all of the following features:
(1a) minor aphthous ulcers, major aphthous ulcers, or herpetiform lesions
(1b) observed by physician or patient
(1c) recur at least 3 times in one 12 month period
(2) 2 or more of the following:
(2a) recurrent genital ulcerations: aphthous ulceration or scarring observed by physician or patient
(2b) eye lesions (anterior uveitis, posterior uveitis, cells in vitreous humor on slit lamp examination, and/or retinal vasculitis observed by ophthalmologist)
(2c) skin lesions:
(2c1) erythema nodosum observed by physician or patient
(2c2) pseudofolliculitis
(2c3) papulopustular lesions
(2c4) acneiform nodules observed by physician in postadolescent patients not on corticosteroid treatment
(2c5) positive Pathergy test
The Pathergy (Behcetine) test:
(1) The physician makes skin needle pricks or an intradermal injection of saline.
(2) It is read by the physician at 24-48 hours afterwards.
(3) A positive reaction is the presence of indurated erythema or pustule formation.
Performance of criteria:
• The sensitivity ranges from 91-95% and specificity 96-98%.
