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Behçet-like Syndromes: A Comprehensive Review.

Gaia Mancuso1, Igor Salvadè1, Adam Ogna2,3

  • 1Allergy and Clinical Immunology Service, Regional Hospital of Locarno, Ente Ospedaliero Cantonale (EOC), 6600 Locarno, Switzerland.

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|January 21, 2026
PubMed
Summary
This summary is machine-generated.

Behçet-like syndrome (BLS) presents with diverse underlying causes, differing from Behçet's disease (BD). Recognizing BLS is crucial for tailored management and understanding BD pathogenesis.

Keywords:
Behçet-like syndromeBehçet’s diseaseHLA-B51autoinflammatory disordersgenetic predispositionimmune dysregulationimmunotherapy

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Area of Science:

  • Rheumatology
  • Immunology
  • Genetics

Background:

  • Behçet-like syndrome (BLS) encompasses Behçet's disease (BD) features associated with distinct conditions like immune defects, infections, or drug exposure.
  • BLS may present differently from BD and is increasingly recognized as a separate clinical entity.
  • Differentiating BLS from primary BD is vital for effective patient management and offers insights into BD pathogenesis.

Purpose of the Study:

  • To systematically review and summarize the clinical features, treatment modalities, and genetic abnormalities reported in Behçet-like syndrome.
  • To consolidate existing knowledge on BLS through a comprehensive analysis of published literature up to January 2024.

Main Methods:

  • A systematic literature search was conducted across PubMed, Scopus, and Embase using relevant keywords for Behçet-like syndrome.
  • Inclusion criteria involved English-language reports of patients over 12 years old with a defined etiology and BD-like manifestations (e.g., recurrent ulcers, gastrointestinal involvement, thrombosis).
  • Data on epidemiology, clinical presentation, laboratory findings, histology, and treatments were extracted and descriptively analyzed.

Main Results:

  • The review identified 100 patients with BLS from 53 publications, with a median age of 44 years and female predominance.
  • Genetic disorders were identified in 70% of cases; common manifestations included skin lesions (68%), fever (56%), and intestinal involvement (43%).
  • Treatments comprised glucocorticoids (65%), DMARDs (32%), biologics (22%), and in some cases, antiviral/antibiotic therapy or chemotherapy.

Conclusions:

  • Various conditions, including monogenic diseases, immune deficiencies, myeloproliferative disorders, infections, and drug reactions, can manifest as Behçet-like features.
  • Clinical presentation and treatment responses vary significantly across different etiologies of BLS.
  • Identifying BLS is essential for appropriate management and advancing the understanding of BD pathogenesis and potential targeted therapies.