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Behçet Syndrome

Laura B. Bishop, MD Reviewed 05/2023
 


BASICS

DESCRIPTION

A chronic, relapsing, multisystem, autoinflammatory disorder. 
  • Precise etiology remains incompletely understood; strong genetic association

  • Clinical manifestations are driven by hypera...

DIAGNOSIS

HISTORY

  • Key symptom: recurrent aphthous stomatitis (RAS) is the most frequent initial presenting symptom (65–70%); present in 97–100% during disease course, occurs in all three clinical subty...

TREATMENT

MEDICATION

Treatment is centered on immunomodulation, with different therapies such as corticosteroids, TNF-α, and phosphodiesterase type 4 (PDE4) inhibitors based on the organ system involve...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Depends on severity of system involvement and medication monitoring

  • Periodic ophthalmologic examinations

DIET

No special diet, although oral ulcers...

REFERENCES

1
Oǧuz  AK, Yılmaz  ST, Oygür  ÇŞ, et al. Behçet’s: a disease or a syndrome? Answer from an expression profiling study. PLoS One.  2016;11(...

CODES

ICD10

  • M35.2 Behcet’s disease

  • N77.0 Ulceration of vulva in diseases classified elsewhere

SNOMED

  • 310701003 Behcet’s syndrome (disorder)

  • 198231000 Ulceration of vulva in Behcet’s disease (disorder)

  • 40344...

CLINICAL PEARLS

  • BS is a clinical diagnosis; utilizing clinical diagnostic criteria are helpful.

  • Consider BS in patients presenting with recurrent oral and genital ulcers.

  • Treatment depends on severity o...

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