BehCET's Disease

Exam Cram

• Behcet's Disease is a rare systemic inflammatory condition or syndrome

• Presentation: mouth & genital ulcers, arthralgia & peripheral neuropathy

• Diagnosis: clinical 

• Management: steroids

Outline

Assessment

Bechet's 1994 Survey %

• Mouth ulcers 100%

• Arthralgia 90%

• Genital ulcers 90%

• Skin lesions 90%

• Eye inflammation 70%

• Pathergy (skin prick test) 30%

• Thrombophlebitis 30%

• Neurological symptoms 30%

• IBD 30%

• DVT 20%

• Headaches, arthralgia, acne

Investigations

• Anti-phospholipid syndrome 25%

Diagnosis

Diagnostic criteria: ICBD 2013 > 3 points

• 2 points

  • Ocular lesions

  • Genital ulcers

  • Oral ulcers

• 1 point

  • Skin lesions

  • Neurological manifestations

  • Vascular manifestations

  • Positive pathergy (skin prick) test

Differentials: Herpes Simplex (HSV) 

Management

Acute & chronic:

1. PO Steroids (eye involvement)

2. DMARDS: azathioprine, cyclophosphamide, etanercept

Complications

• Anterior uveitis

• Stroke

References and Resources

1. https://behcetsuk.org/how-is-behcets-diagnosed/

2. https://www.nhs.uk/conditions/behcets-disease/#:~:text=Beh%C3%A7et's%20disease%2C%20or%20Beh%C3%A7et's%20syndrome,a%20number%20of%20other%20conditions).

Written 2024.