Behçets

Behçet's disease, also known as Behçet disease, is a chronic relapsing inflammatory disorder characterized by mucocutaneous and systemic manifestations. It is named after Hulusi Behçet, who described its triad of recurrent oral ulcers, genital ulcers, and uveitis in 1937. The disease shows the highest reported prevalence along the historic Silk Road, with regional variation; prevalence is generally higher in Turkey and other parts of the Mediterranean, Middle East, and East Asia.

Clinical features commonly begin with recurrent oral aphthous ulcers and genital ulcers. Eye involvement, including anterior or posterior uveitis and retinal vasculitis, can occur and may threaten vision. Skin findings include erythema nodosum–like lesions and papulopustular eruptions. Joint involvement is frequent and usually nonerosive. Less commonly, vascular, neurological, gastrointestinal, and cardiac complications may arise. The disease course is typically relapsing and remitting, with symptom patterns varying between individuals and over time.

Pathophysiology is not fully understood, but it is considered a complex immune-mediated condition with genetic susceptibility, notably a strong association with HLA-B51 in many populations. Environmental and infectious triggers may contribute. Pathergy, a hypersensitivity reaction to minor skin trauma, is observed in a subset of patients.

Diagnosis relies on clinical criteria rather than a single laboratory test. Diagnostic tools include criteria sets such as the International Criteria for Behçet's Disease and other regionally used frameworks, along with documentation of recurrent mucocutaneous ulcers and organ involvement. Treatment is tailored to organ involvement and severity, ranging from topical therapies for mucocutaneous lesions to systemic immunosuppressants and biologic agents for ocular, vascular, or neurological disease. Multidisciplinary care is common, and prognosis varies with the extent and location of organ involvement.