Behcets disease

Diagnosis: Behcets disease

Behcets disease

Clinical Presentation

Behcets disease

Clinical History

Recurrent oral aphthous ulcers (≥3 times/year), genital ulcers, ocular inflammation. Pathergy test. Demographics: Silk Road populations.

Treatment

Oral/genital ulcers: topical corticosteroids, colchicine. Severe mucocutaneous: apremilast, azathioprine, thalidomide. Ocular: azathioprine, infliximab, adalimumab, interferon-alpha. Vascular: immunosuppressives + anticoagulation.

Differential Diagnosis

• Herpes simplex • Aphthous stomatitis • Crohn disease • Reactive arthritis • SLE • Sweet syndrome • Syphilis

Key Learnings

• Systemic vasculitis affecting arteries and veins of all sizes • Classic triad: recurrent oral ulcers, genital ulcers, ocular inflammation (uveitis) • Pathergy test: sterile pustule 24-48h after needle prick — pathognomonic (especially in endemic populations) • Silk Road disease: Turkey, Iran, Japan, Korea — HLA-B51 association • Oral ulcers are required for diagnosis (International Study Group criteria) • Most feared complication: ocular disease → blindness if untreated

Tags: Behcets disease, case, dermatologist, dermatology, kodachrome