Prolonged fever with erythema nodosum-like lesions: Behçet's disease as a diagnostic challenge in a tropical low-prevalence setting

Behçet's disease (BD) is a multi-system inflammatory disorder characterised by recurrent oral, genital or ocular inflammation, and may present with variable-vessel vasculitis. 1 It has a distinctive geographic distribution, with the highest prevalence along the historic Silk Road, particularly in Turkey and Iran. 2 – 4 In contrast, BD is considered rare in India, with limited epidemiological data and predominantly hospital-based series. 5 , 6 Although recurrent oral ulcers are nearly universal, systemic manifestations, including ocular, vascular, neurological and gastrointestinal involvement, vary across regions. 2 , 3 Fever is not a classic presenting symptom in mucocutaneous disease, but may occur in vascular or neuro-Behçet's, where it reflects heightened systemic inflammatory activity. 7 Consequently, BD may masquerade as a prolonged febrile illness, particularly before characteristic features emerge. Atypical presentations may delay diagnosis, particularly in low-prevalence regions.