A previously healthy 25 year-old Spanish woman presented with two days of fever, bilateral erythematous axillary swelling and tender vulvar and labial lesions. She had no known medical problem and was sexually active with a male partner who had a history of oral herpes simplex virus (HSV) lesions. She had no family history of autoimmune or vasculitic diseases. The initial examination revealed crusting ulcerative lesions in the labia majora, vaginal discharge and tender axillary indurations. The patient was started on acyclovir for presumed disseminated HSV.
Within 24 hours of admission, the patient developed persistent fever associated with neck pain and headache. The axillary lesions evolved into multiple acneiform pustules; additional similar lesions appeared on the back. She also developed new tender maculopapular lesions on the arms and legs most consistent with erythema nodosum. Antimicrobials were empirically broadened against bacterial meningitis and HSV encephalitis. A lumbar puncture revealed pleocytosis (218 cells/mm3) with 91% neutrophils, normal glucose and mildly elevated protein. All other infectious workup remained negative, including cultures of the acneiform skin lesions and HSV polymerase chain reaction of the serum, cerebrospinal fluid and vulvar lesions. Pelvic ultrasound did not reveal signs of pelvic inflammatory disease.
On hospital day six, the patient reported bilateral arm swelling, and a duplex ultrasound revealed deep vein thrombosis of the left axillary vein and multiple bilateral superficial venous thromboses. Erythrocyte sedimentation rate peaked at 51 mm/hr and C-reactive protein at 188 mg/L. A preliminary diagnosis of neuro-Behcet’s disease was made. The patient was started on intravenous methylprednisolone and oral colchicine with dramatic resolution of the fever, headache and cutaneous findings. She was also started on anticoagulation, and MRI of the brain ruled out venous sinus thrombosis. A serum HLA-B51 titer returned positive after her hospital discharge. The patient’s course was notable for negative pathergy tests and the absence of oral ulcers or ocular involvement.
Although its diagnosis often relies on the presence of oral ulcers, about 3% of patients with Behcet’s disease do not have recurrent oral ulcers at the time of diagnosis. While the presence of HLA-B51 does not confer much diagnostic utility in areas with a low disease prevalence such as the United States, HLA-B51 positivity occurs in greater than 55% of Behcet’s disease patients with central nervous system involvement.
The thorough and timely infectious workup along with an interdisciplinary approach involving consultative services, such as infectious disease and rheumatology, led to the prompt diagnosis and treatment of neuro-Behcet’s disease.