Answer to CC #7

Case Challenge #7 presented a 26 year old Asian male with his 3rd episode of headache and neck stiffness associated with uveitis, oral ulcers, and genital ulcers. Labs revealed a leukocytosis and CSF with 45 cells (85% Lymphs), protein of 65, normal glucose, a negative gram stain.

We asked which of the following would be most compatible with the most likely diagnosis?

The results of the voting are as follows:

Behcet Results
The correct answer is:

Pustule at the site of a blood draw, or (Pathergy!)

This patient has Behçet’s disease, a multisystem inflammatory disease marked by:
  • Recurrent oral aphthous ulcers and at least 2 of the following:
    • Recurrent genital ulcers
    • Ocular lesions: uveitis or hypopyon
    • Pathergy (pustule at site of needle stick)
  • Ethnicity: Asian or Eastern Mediterranean descent
  • Also associated with GI disease and recurrent aseptic meningitis
  • Rx: Colchicine

As seen in our previous post, pathergy phenomenon is when a small red bump or pustule occurs 1 to 2 days after a forearm is pricked with a sterile needle. This test is often associated with Behcet’s disease having a low sensitivity but high specificity for the disease, as a positive test supports a diagnosis of Behcet’s and is not definite. This past week’s NEJM had a case of a patient with newly diagnosed Behcet’s disease and a positive pathergy test. Click on the links below to read the article from NEJM and learn more from the Hopkins Vasculitis Center.

The Johns Hopkins Vasculitis Center – Behcet’s Disease

A 25-Year Old Man with Oral Ulcers, Rash, and Odynophagia 

Thanks for playing, case challenge #8 will be posted next week!