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Behçet Disease: An Update for Dermatologists.
Erkan Alpsoy1, Burcin Cansu Bozca2, Asli Bilgic2
1Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059, Antalya, Turkey. ealpsoy@akdeniz.edu.tr.
American Journal of Clinical Dermatology
|June 1, 2021
Summary
Behçet disease (BD) is a chronic vasculitis with diverse organ involvement. Understanding its genetic basis and immune triggers aids in developing new treatments like apremilast and anti-TNF-α agents for improved patient outcomes.
Area of Science:
- Rheumatology
- Immunology
- Genetics
Background:
- Behçet disease (BD) is a chronic, relapsing systemic vasculitis with unknown etiology.
- Characterized by oral/genital ulcers, skin lesions, and potential ocular, articular, vascular, GI, neurological, and cardiac involvement.
- Prevalence is higher along the historical Silk Road, with universal distribution.
Purpose of the Study:
- To summarize the pathogenesis, clinical manifestations, and current management of Behçet disease.
- To highlight the role of genetic factors and immune system dysregulation in BD.
- To discuss the impact of novel therapies on patient prognosis and quality of life.
Main Methods:
- Review of existing literature on Behçet disease.
- Analysis of genetic associations, particularly HLA-B51 and ERAP1.
- Evaluation of current and emerging therapeutic strategies.
Main Results:
- Mucocutaneous manifestations are key diagnostic hallmarks.
- Genetic factors (HLA-B51) and immune responses (Th1/Th17, neutrophils) are central to pathogenesis.
- New drugs like apremilast and anti-TNF-α agents show efficacy.
Conclusions:
- BD pathogenesis involves complex genetic and immune interactions.
- Early intervention is crucial to prevent irreversible organ damage.
- Novel therapies offer improved quality of life and survival for BD patients.
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