Behçet's disease (BD), a chronic multi-systemic disorder, presents diverse clinical manifestations depending on patient ethnicity and geographic region. Use of varying diagnostic criteria augments clinical heterogeneity. We aimed to characterize heterogenous manifestations in patients with full-blown BD fulfilling the major diagnostic criteria in use. We retrospectively analyzed 338 patients diagnosed with complete BD based on Japanese diagnostic criteria, which fulfill both International Study Group (ISG) criteria and the International Criteria for BD (ICBD). Unbiased clustering analysis was performed to elucidate the heterogeneous spectrum, followed by subgroup analysis of identified clusters. Results of unbiased clustering analysis identify dominant skin lesion type as an important factor that determines clustering among the heterogenous BD patients. Regression analysis reveals that presence of predominantly papulopustular lesions has protective effect for vascular involvement compared to other skin phenotypes. In conclusion, unbiased clustering analysis highlights that dermatologic manifestation can be a factor to understand the heterogeneity of BD and determining the dominant type of skin lesions may help clinicians predict major vascular involvement.
Keywords: Behçet's disease; clinical manifestation; papulopustular lesions; skin manifestation; vasculitis.
© 2021 Japanese Dermatological Association.