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Internal Medicine

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You are consulted to see a 26-year-old man who was admitted yesterday with fever, headache, and a mildly stiff neck. In the emergency room, he had an LP and then was immediately given ceftriaxone, vancomycin and dexamethasone for suspected bacterial meningitis. The CSF Gram stain was negative; there were 128 white blood cells of which half were neutrophils and half were mononuclear cells; glucose was normal; protein was 72.

Today, blood and CSF cultures were reported to be negative; the patient was not clinically improved.

The patient came to the United States from Japan a few months ago to attend graduate school.

On your exam, you note three aphthous ulcers on the soft palate, and the patient says he has had “fever blisters” intermittently for years. He also reports that he has had two episodes of painful penile ulcers but is quick to deny any sexual contact.

His illness is most likely to respond to which one of the following?

  • Ribavirin

  • Acyclovir

  • Ganciclovir

  • Colchicine

This patient’s illness is most consistent with a diagnosis of Behçet’s Disease, a multisystem inflammatory disease characterized by recurrent painful aphthous oral ulcers, recurrent painful genital ulcers, and eye or skin lesions. Pathergy, development of a red papule or pustule more than one-half centimeter in diameter is often seen one to two days after skin trauma, such as a needle stick. Behçet’s is particularly prevalent in persons of Asian or Eastern Mediterranean descent. Aseptic meningitis, as in this patient, may also occur, as can GI symptoms.

Colchicine has long been the drug of choice for Behçet’s Disease, although is best studied for mucocutaneous disease. Other agents that have been used for treatment include dapsone, corticosteroids, thalidomide, and immunosuppressive agents. Anakinra has been used in refractory cases.

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