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