Case Presentation:

This is a 13 year-old premenarchal girl with no significant past medical history who began to experience malaise, headaches, and subjective fevers 5-6 days prior to admission. Subsequently she began to experience vulvar pain. This was followed by vulvar erythema which developed into “black dots” that began to ulcerate. Two days prior to admission, she noted a targetoid rash on her left shoulder, which had been preceded by a non-engorged tick bite on that extremity 2 weeks earlier. Subsequent evaluation by her pediatrician resulted in initiation on doxycycline, but her vulvar pain continued to worsen. Dysuria increased to the point of urinary retention, resulting in admission. Her physical examination was significant for being afebrile and having two 0.5×1 cm shallow ulcers with yellowish exudate over the left labia majora, which was mildly erythematous. Her left shoulder manifested a circular blanching erythematous rash 20 cm wide with central clearing. Initial laboratory findings were significant for lack of leukocytosis, mild microcytic anemia with hemoglobin of 10.8 g/dL, ESR of 37 mm/hr, and unremarkable serum chemistries and liver panel. Based on the concern over Lyme disease and related vaginal ulceration, she was continued on oral doxycycline and treated symptomatically for vulvar pain with oral oxycodone and topical lidocaine. Two days after admission she was discharged with symptomatic improvement and some visible improvement in the vulvar ulcerations. After discharge, Lyme disease IgM Western blot came back positive with negative IgG Western blot. EBV viral capsid IgM also came back positive at 57.0 with negative IgG and nuclear antigen antibody. The remainder of her workup, including GC/chlamydia urine probe, RPR, and HIV were negative.

Discussion: Nonsexual acute genital ulceration (NAGU), also known as Lipschutz ulcers, are a rare complication of Lyme disease, but has also been associated with EBV, Mycoplasma, HIV, and influenza disease. To date, only one prior case in the literuature of Lipschutz ulcers has been confirmed as being caused by Lyme Disease. The clinical history and laboratory findings in this case would suggest that the etiology is Lyme disease, but EBV remains a possibility. The differential also includes inflammatory disease (e.g., Behcets disease) and sexually transmitted disease, but the low ESR and negative STI testing make these less likely. Lyme and EBV antibodies are known to cross-react, and follow up seroconversion of IgG will help to clarify the diagnosis.

Conclusions: In areas where Lyme disease is endemic, patients with acute  non-sexual genital ulceration should be evaluated for Lyme disease. EBV, mycoplasma, influenza, CMV and inflammatory conditions should also be considered.