Behçet's disease
Overview
Definition & Demographics
- Chronic, inflammatory, relapsing generalized vasculitis with multiple organ involvement.
- Etiology: Classically, it is autoimmune + HLA-B*51 (+ viral triggers).
- Manifestation: 20–40 years of age, men more frequently, Silk Road population (Turkey, Iran, Mediterranean, East Asia) — highest prevalence.
- Histology: neutrophil-rich perivascular inflammation with leukocytoclasia, affecting small venous mucocutaneous vessels.
Clinical Presentation (ISG Criteria 1990 / ICBD 2014)
- Recurrent oral aphthae obligatory (≥ 3 ×/year) PLUS ≥ 2 others:
- Genital aphthae / ulcers
- Eye involvement (anterior / posterior uveitis, hypopyon, retinal vasculitis)
- Skin manifestations: Erythema nodosum-like nodules, pseudo-folliculitis, pyodermas, sterile pustules, thrombophlebitis migrans
- Pathergy test positive (sterile pustule 24–48 h after needle prick)
- Systemic: Arthritis, GI vasculitis, CNS involvement (Neuro-Behçet), pulmonary artery aneurysms, large vein thromboses.
Clues for Behçet's vs. Recurrent Aphthous Stomatitis
- Posterior oral cavity affected (in recurrent aphthous stomatitis usually only anterior two-thirds).
- Very large, strikingly configured aphthae (not simply round).
- General symptoms with fever, night sweats, weight loss, ESR ↑.
Diagnostics
- CBC + Diff + ESR + CRP
- Pathergy test (sterile needle 4 × into forearm skin → reading 24–48 h)
- HIV in case of risk factors
- Ophthalmological consultation obligatory (visual acuity, fundus)
- HLA-B*51 supportive, not obligatory
- MRI of the skull in case of neurological symptoms
Differential diagnoses
- Minor aphthous ulcer
- Secondary syphilis (lues II)
- Herpes simplex labialis
- Reactive arthritis (Reiter syndrome)
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In the DermaFuchs app: 1 flashcards · 2 clinical images
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Note: This page is intended for medical education and does not replace diagnosis or treatment decisions in individual cases. Treatment and follow-up content is available in the app.