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Behçet Syndrome

Abdul Aleem, MD and Hiral Shah, MD, FASGE, FAGA, FACG Reviewed 06/2019
 


BASICS

DESCRIPTION

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

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

    • C...

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, and treatm...

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(2):e0149052. [View Abstract on OvidMedline]
2
Bulur...

ADDITIONAL READING

  • Hamuryudan V, Hatemi G, Tascilar K, et al. Colchicine in Behçet syndrome: a longterm survey of patients in a controlled trial. J Rheumatol.  2014;41(4):735–738. [View Abstract ...

CODES

ICD10

  • M35.2 Behcet’s disease

  • N77.0 Ulceration of vulva in diseases classified elsewhere

ICD9

  • 136.1 Behcet’s syndrome

  • 616.51 Ulceration of vulva in diseases classified elsewhere

SNOMED

  • 310701003 Behcet’...

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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