Cutaneous and Extracutaneous Manifestations of Behçet's Disease Linked to Its Disease Activity and Prognosis.

Abstract

BackgroundBehçet’s disease is a potentially fatal vasculitis disorder involving vessels in both the arterial and venous systems. Cutaneous manifestation is the most common sign in Behçet’s disease, but its relation to disease activity and prognosis is lacking.ObjectiveOur study aims to determine the relationship between cutaneous, extracutaneous manifestations with Behçet’s disease activity, morbidity, and mortality.Materials and MethodsPatients diagnosed with Behçet’s disease were identified by using the 1990 International Study Group for Behçet’s disease criteria and/or the 2014 international criteria for Behçet’s disease. Data regarding patients’ clinical manifestations, laboratory results, disease activity, and prognosis were retrieved and analyzed. Disease activity was evaluated using the 2006 Behçet’s disease current activity form (BDCAF).ResultsOf 119 patients, the mean age at diagnosis was 35.7 ± 10.7 years and 60% were female. Median disease duration was 96 months (IQR 48–168). Minor oral ulcer at the buccal mucosa was associated with lower median BDCAF score (p = 0.003), whereas minor oral ulcer at the tonsil was related to higher BDCAF score (p = 0.024). Male gender was associated with higher cumulative dose of corticosteroids (p = 0.003) and hospitalization (OR = 2.89 (95% CI [1.10, 7.57]), p = 0.031). Ocular manifestations were related to higher dose of corticosteroids (p = 0.002) and morbidity (OR = 4.39 (95% CI [1.57, 12.29]), p = 0.005).ConclusionOur study suggests that different locations of minor oral ulcers help predict disease activity. Male gender and the presence of ocular manifestations resulted in less favorable outcomes for Behçet’s disease.