Related Experiment Video
Updated: Aug 24, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Behçet's disease
1Wright Fleming Institute, Imperial College School of Medicine, Norfolk Place, London W2 1PG, UK. s.marshall@imperial.ac.uk
Insights
Behçet
Area of Science:
- Rheumatology
- Immunology
- Genetics
Background:
- Behçet's disease is a systemic vasculitis with unknown etiology.
- It presents with recurrent oral/genital ulcers and ocular inflammation, affecting multiple organ systems.
- Genetic predisposition, particularly HLA-B*51, plays a role in disease pathogenesis.
Purpose of the Study:
- To review recent advancements in understanding Behçet's disease pathogenesis.
- To discuss current clinical diagnostic criteria and their effectiveness.
- To evaluate the evidence base for established and novel therapeutic agents.
Main Methods:
- Literature review focusing on recent developments in Behçet's disease.
- Analysis of diagnostic criteria and their clinical application.
- Evaluation of clinical trial data for existing and emerging treatments.
Main Results:
- The leading hypothesis suggests an inflammatory response to an infectious agent in genetically susceptible individuals.
- The 1990 classification criteria are clinically effective for diagnosis.
- Cyclosporin, azathioprine, and anti-tumour necrosis factor therapies show efficacy in specific clinical contexts.
Conclusions:
- Understanding Behçet's disease pathogenesis is evolving, with a focus on genetic and environmental triggers.
- Clinical diagnosis relies on established criteria, supported by ongoing research.
- Evidence supports the use of immunosuppressants and biologics, with further research needed for optimal therapeutic strategies.
Abstract:
Behçet's disease is a systemic vasculitis characterized by recurrent oral and genital ulcers, and ocular inflammation, and which may involve the joints, skin, central nervous system and gastrointestinal tract. It is most common in those of Mediterranean and Eastern origin, although it also affects Caucasians. The aetiology of the disease remains unknown, but the most widely held hypothesis of disease pathogenesis is that of a profound inflammatory response triggered by an infectious agent in a genetically susceptible host. Supporting this is the consistent association of disease susceptibility with polymorphisms in the human leukocyte antigen complex, particularly HLA-B*51. The diagnosis is a clinical one, and although there is no single laboratory test specific for the diagnosis of Behçet's disease, the 1990 classification criteria perform well in a clinical context. Whereas many favoured treatments for single or multisystem disease still lack a sound evidential base, cyclosporin and azathioprine perform well in clinical trials, and evidence is accumulating for the efficacy of anti-tumour necrosis factor therapy in particular clinical situations. This review will focus on recent developments in the understanding of disease pathogenesis and clinical diagnosis, and review the evidence base for both established and new agents in the therapeutic strategy.
Related Concept Videos
Skin Diseases and Disorders
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
Giardiasis
Hypersensitivity Reactions: Delayed Hypersensitivity Reactions
Trichomoniasis
Acne Infection
Graves' Disease I: Introduction