An 18-year-old woman presented with intermittent fevers, vulvodynia, and vaginal ulcers for five days. She initially went to an urgent care center after two days of fevers associated with vaginal irritation and two small ulcers on her labia. Tests for the herpes simplex virus were negative, and she was told she had a viral syndrome and to use Desitin for ulcer pain.
Three days later, she came to our ED when her vaginal pain became intolerable, and she noticed the ulcers expanding. She had associated symptoms that progressed to fevers, myalgias, mild headache, nausea, and decreased appetite. She did not have oral ulcers, difficulty swallowing, abdominal pain, dysuria, or vaginal discharge. She said she had never been vaginally or orally sexually active.
Two years before, she had had slightly painful vaginal ulcers, and was started on acyclovir. Her HSV test at that time was also negative. Accutane was the only medicine she was taking, having started it a month earlier, but she had not taken it for the past week. She reported allergies to amoxicillin and codeine and that her mother had eczema and her father had hypertension and neuropathy.
Her vital signs were a temperature of 38.8°C, a blood pressure of 132/66 mm Hg, a pulse of 94 bpm, a respiratory rate of 18 bpm, and oxygen saturation of 99% on room air. She was alert and oriented to person, place, and time.
She had no nuchal rigidity, oral lesions, cervical lymphadenopathy, skin lesions, or rashes. Her abdomen was soft and not distended or tender. Her pelvic exam showed extensive bilateral ulceration of the external labia minora and the inner portion of the labia minora extending into the vaginal mucosa. The largest ulceration was dusky with purulent drainage.
What are your differential diagnoses? What diagnostic workup is needed?
Find the diagnosis and case discussion on the next page.
Diagnosis: Lipschütz Ulcer from Acute Epstein-Barr Virus
Lipschütz ulcer, or acute vulvar ulceration, is an uncommon disease that occurs in sexually inactive young women. It was first described in 1912 by the Austrian dermatologist and microbiologist Benjamin Lipschütz, who published four cases of girls aged 14-17. (Arch Dermatol Syph. 1913;114:363.) The diagnosis is a clinical one of exclusion based on history and physical. Patients presented with acute painful genital ulcers and fever.
Lipschütz ulcer is also known as reactive nonsexually-related genital ulcers. The disease manifests as self-limited ulcers in response to an acute illness, commonly Epstein-Barr virus (EBV) or other upper respiratory infections. (J Am Acad Dermatol. 2010;63:44; J Am Acad Dermatol. 2004;51:824.) Often the cause is not determined.
The pathogenesis of the acute genital ulceration is unclear, but it is thought to be a hypersensitivity reaction to a viral or bacterial infection leading to deposition of immune complexes in dermal vessels with associated inflammation, ischemia, necrosis, and finally ulceration. (UpToDate. Acute genital ulceration [Lipschütz ulcer]. 31 Oct 2018; http://bit.ly/2XpFIls.)
Her physical exam showed deep ulcers larger than 1 cm, necrotic with grayish exudate or adherent gray-black eschar and red violaceous borders. These ulcers are typically symmetrical, often described as kissing lesions, and involve the labia minora but can extend to the labia majora, perineum vestibule, and lower vagina. Associated signs include labial edema and inguinal lymphadenopathy.
Most patients also report prodromal influenza or mononucleosis-like symptoms with fever, malaise, and pharyngitis. This is not a sexually transmitted infection, so no risk of spreading it exists. Spontaneous healing is complete in two to six weeks, usually without scarring. Approximately 30-50 percent of patients will experience recurrent episodes and develop complex aphthosis. (UpToDate. Acute genital ulceration [Lipschütz ulcer]. 31 Oct 2018; http://bit.ly/2XpFIls.)
Differential diagnoses for genital ulcers are sexually transmitted infections or noninfectious diseases that cause genital or orogenital ulcerations such as Behcet's syndrome, Crohn's disease, pyoderma gangrenosum, lichen planus, pemphigus, complex aphthosis, and drug eruptions. (Dermatol Ther. 2010;23:533; UpToDate. Approach to the patient with genital ulcers. 5 Dec 2018; http://bit.ly/2IH9mPl.) Viral etiology such as EBV, mononucleosis, or influenza should also be considered.
Workup should include HSV polymerase chain reaction or viral culture swabs of the ulcer. Syphilis serology should be considered, and EBV IgM and IgG serology are indicated. A complete blood count with differential to assess for lymphocytosis and liver function tests should be performed when considering a previous acute viral infection that led to genital ulceration. Bacterial cultures of the ulcer should be collected if there are concerns for bacterial superinfection or vulvar cellulitis. Biopsies are often not required unless a specific skin disease is suspected.
Treatment is primarily supportive, and consists of pain control (topical and systemic), local hygiene, and wound care to prevent subsequent bacterial infection. Hospitalization with Foley catheterization might be needed for severe pain and to avoid urinary retention. (Dermatol Clin. 2010;28:753.)
The patient in this case continued to be in severe distress because of her extensive lesions, and she was unwilling to urinate due to severe vulvodynia. She was given topical lidocaine, parenteral NSAIDs, and opioids for pain with slight temporary relief. Gynecology was consulted in the emergency department, and the patient was admitted to the hospital for further pain control. The patient was offered a Foley catheter for comfort but declined in favor of sitz baths and perineal irrigation for cleansing. Given the fever and purulent drainage, she was started on vancomycin, ceftriaxone, and Flagyl for superimposed skin infection.
No antivirals were given because her HSV PCR was normal. Her EBV IgM and negative EBV IgG were positive, indicating acute EBV infection. Dermatology and rheumatology were consulted during the admission, and her physicians concluded that the diagnosis was Lipschütz ulcer related to EBV. The patient was also started on topical steroids (clobetasol propionate 0.05% ointment twice daily) and topical estrogen.
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Dr. Selbyis an assistant professor of emergency medicine at the University of Colorado School of Medicine and the medical director of the forensic nursing program at University of Colorado Hospital in Aurora. Follow her on Twitter@DocSelbs. Read her past columns athttp://bit.ly/EMN-QuickConsult.