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From the American College of Rheumatology

Rheumatology for Primary Care
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Behcet’s Syndrome Case Studies

Home ยป Behcet’s Syndrome Case Studies
Behcet’s Syndrome Case Studies
  • Case 1

  • Case 2

  • Case 1

A 35 yo M of Turkish descent presents to clinic with oral ulcers.

History & Physical Exam

Patient Reports
  • Oral ulcers started about 1 year ago and have been recurring every 2-3 months since then. 
  • He has had one genital ulcer during this time. He is sexually active with one partner and uses barrier protection. 
  • He had laboratory work drawn about 4 months ago and noticed increased redness at the site of the needlestick, which persisted for about 48 hours (he notes this has been occurring since childhood). 
  • Does not take any medications other than occasional OTC ibuprofen for headaches.
  • The ulcers have started to become more bothersome, and he would like to see if there is anything he can do to help with their recurrence. 
Physical Exam
  • Two ulcers in his mouth
  • Vital signs are normal.
  • Labs are unremarkable.
Differential Diagnosis

Next Steps & Treatment

  • The report of pathergy would need to be verified and visualized by a physician.
  • At this time, no treatment should be started until infectious etiology is ruled out. 
  • If no other cause is identified, consider a topical steroid (for his oral ulcers – a dexamethasone elixir, for genital ulcers – a topical steroid), and if uncontrolled or for prevention of recurrent ulcers, colchicine or apremilast can be considered.
  • Rheumatology follow-up is warranted. 
  • Case 2

A 45 yo Japanese M with history of Crohn’s disease had recent colonoscopy, which revealed increasing and worsening ulcers in his colon.

Clinical Presentation & History

  • Biopsy of the new ulcers revealed nonspecific inflammation with no granulomas.  
  • Currently on treatment with anti-TNF alpha inhibitor and had good control of his symptoms until last year. 
  • He has noticed occasional oral ulcers that have increased while he has been on immunosuppressives, which he attributed to his history of oral herpes. 
  • He was diagnosed with Crohn’s disease at 25 yo, which was confirmed with bx with granulomas. 
  • Persistent redness in the area of needle sticks as child and adult.  
  • He recently had evaluation by an ophthalmologist; diagnosed with uveitis and treated with topical eye drops

Physical Exam & Tests

  • His vital signs today are normal.
  • Physical exam is unremarkable. 
  • CBC, CMP, ESR, and CRP are all within normal limits.
Differential Diagnosis

Treatment

  • Alternative or additional immunosuppressant if Behcet’s is active or current anti-TNF has become ineffective

Differential Diagnosis

This patient is a male in his 20s-40s from Eastern European/Mediterranean descent with recurrent oral ulcers and suspected pathergy. Behcet’s should be in differential; however, infectious etiology needs to be ruled out, as oral and genital ulcers can also be due to other infectious causes, such as STDs.

Differential Diagnosis

  • GI involvement of Behcet’s is more common in Japan (25-30%) and is characterized by gastrointestinal ulcerations.
  • Crohn’s disease also presents with ulcers, which can cause confusion regarding diagnosis. Crohn’s disease usually has granulomas present on biopsy, while Behcet’s does not. 
  • The patient’s recent change in bowel habits, increasing ulcerations in colon (which were recently well controlled on TNF-inhibitor), increasing oral ulcers, and history of pathergy with diagnosis of uveitis raise suspicion for another etiology of his current symptoms. 
  • His ethnicity and prior autoimmune disease (Crohn’s) put him at risk of Behcet’s syndrome. 
  • His worsening oral ulcers may be a Behcet’s manifestation and not related to herpes labialis as initially thought. However, this would need to be investigated, and he would need to be evaluated while he has an ulcer outbreak.
  • His pathergy would also need to be confirmed by a physician.

Related Links

Behcet’s Syndrome Overview

 

ACR Vasculitis Treatment Guideline

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Primary Care2024-05-21T13:43:20+00:00

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