MUCOCUTANEOUS CRITERIA FOR THE DIAGNOSIS OF BEHCETS-DISEASE - AN ANALYSIS OF CLINICOPATHOLOGICAL DATA FROM MULTIPLE INTERNATIONAL CENTERS

Background: Although four of five of the new international criteria fo r the diagnosis of Behcet's disease relate to mucocutaneous lesions, d isagreement exists as to the exact nature of cutaneous lesions (e.g., vessel-based vs follicular). Objective: Our purpose was to review clin ical data, clinical photographs, and skin biopsy specimens from multip le medical centers throughout the world to monitor current practice in the implementation of mucocutaneous diagnostic criteria for Behcet's disease. Methods: Ten medical centers responded to a request to collab orate by sending clinical data, photographs of cutaneous lesions, and biopsy specimens from 22 patients. Results: Of specimens from 22 patie nts, 14 revealed a histopathologic pattern of neutrophils containing p erivascular and interstitial inflammation, whereas specimens from thre e patients revealed only mononuclear cells in a vessel-based pattern. Biopsy specimens from three patients revealed primarily folliculocentr ic inflammation and an additional two specimens were from erythema nod osum-like lesions. Conclusion: Perivascular inflammation was the predo minant histopathologic finding in specimens of cutaneous lesions in th is clinical series. Folliculocentric lesions could not be predicted on the basis of review of clinical photographs. Histopathologic assessme nt of cutaneous lesions is crucial if the proposal is accepted that ex clusion of folliculocentric lesions is important to ensure accurate im plementation of diagnostic criteria in patients with suspected Behcet' s disease.