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Behçet Syndrome

Laura B Bishop, M.D. and Ariel Carpenter, M.D. Reviewed 06/2022
 


BASICS

DESCRIPTION

  • Behçet syndrome (BS) represents a chronic, relapsing, multisystem, autoinflammatory disorder.

    • Precise etiology remains incompletely understood; strong but diverse genetic component

    • Cli...

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

Due to its complex pathogenesis, geographical differences in its clinical presentation, there are currently no widely accepted treatment recommendations for managing BS. Treatment ...

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(...

ADDITIONAL READING

Saygin  C, Uzunaslan  D, Hatemi  G. Currently used biologic agents in the management of Behçet’s syndrome. Curr Med Chem.  2015;22(16):1976–1985. [View Abs...

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.

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

  • Treatment depends on severity of disease and medication side effect profile.

  • Refer e...

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