Patient Presentation
A 12-year-old female came to the emergency room with painful urination for 2 days. The pain was increasing in intensity and was persistent. It was worse with urination. Her mother had seen a “red spot” in her vaginal area and they had tried to apply petroleum jelly and use acetaminophen without much relief. She had a temperature of 100.2°F and was more tired.

The past medical history was negative for any gynecological problems or urinary tract infections. She also denied any sexual contact or abuse. She denied any trauma, chemical irritant contacts or new soaps/lotions/etc. She had recently recovered from COVID-19, and denied any other illnesses. She had no systemic illnesses and the family history was negative for any autoimmune problems other than Type 2 diabetes. The review of systems was otherwise negative.

The pertinent physical exam revealed overall, a well-appearing female who was in some discomfort. Her vital signs and growth parameters were normal.
Her general physical examination was normal and her genital examination showed ulcerated lesions on the labial minora that were mirror images. They were about 8-12 mm in size and were deeply erythematous with “punched out” edge. There was no vaginal discharge and the hymen appeared intact. The perineum and anus were normal.

The laboratory evaluation included a urinalysis which was normal. The diagnosis of acute labial ulcerations was made. Given that she was sexually-naive and had a recent illness the most likely cause was a Lipschultz ulcer. As the patient had increasing pain, topical lidocaine was prescribed with followup with her regular doctor. Emollients and sitz baths were also recommended along with continued use of acetaminophen. The patient’s clinical course 4 days later revealed that the pain was better controlled and she overall was feeling better. Her mother had looked at the lesions and felt that they were unchanged.

Discussion
Acute genital ulcers (AGU) are much less common in sexually-naive women than sexually experienced or active women. The differential diagnosis of AGU is large. For sexually active women Herpes simplex virus is the most common cause. Sexually transmitted infections are also included in this differential. In addition to the pain, AGUs can cause distress for the patient and family as possible sexual abuse must be considered.

The differential diagnosis of AGU in non-sexually active women includes:

• Aphthosis
  • Lipschutz ulcer
  • Idiopathic
• Autoimmune or inflammatory diseases
  • Behcet disease
  • Bullous pemphigoid
  • Inflammatory bowel disease
  • MAGIC syndrome
  • Pemphigus vulgaris
  • Pyoderma gangrenosum
• Infection
  • Epstein Barr virus
  • Cytomegalovirus
  • Influenza A
  • Mumps
  • Mycoplasma
  • Paratyphoid
  • Salmonella
  • SARS-CoV-2 (COVID 19) – possibly?
• Medication
  • Fixed drug eruption
  • Non-steroidal anti-inflammatory drugs
• Trauma
  • Irritation, chronic
  • Trauma

Learning Point
Lipschutz ulcers (also known as ulcus vulvae acutum) were described in 1913 by Benjamin Lipschutz an Austrian microbiologist and dermatologist. They are ulcerations in young adolescents without a history of sexual activity. The painful acute ulcers are described as “…deep, necrotic with a purplish single or multiple margins, presenting in a mirror [kissing] pattern, and primary affect the medial face of the labia minora.” Fever and malaise commonly occur with it and other signs and symptoms can occur including adenopathy, headache, myalgia and pharyngitis. Symptoms can last for some weeks. The cause is not known but it has been associated with infections such as Epstein Barr virus and other infectious causes. It is early in the COVID-19 epidemic and it is possible that this is also a new cause.

Lipschutz ulcer actual incidence and prevalence is unknown but felt to be underdiagnosed. A 2019 retrospective case review of women (N=232) seeking gynecological care for acute genital ulcers found that ~4.4% had possible Lipschultz ulcer (which included patients < 20 years old) and if no age restrictions were in place then the number increased to ~35.9%.

Some consider juvenile gangrenous vasculitis of the scrotum as the male counterpart for Lipschutz ulcer.

Evaluation of AGU depends a great deal with the history, including history of autoimmune symptoms, sexual activity, and acute infectious symptoms and evaluation should be tailored to the patient. Patients without a history of sexual activity and negative autoimmune or infectious symptoms may not need an extended evaluation and idiopathic ulcers should be considered. Treatment for Lipschultz ulcers includes hygiene with sitz bath or whirlpool debridement, and pain control including topical medications and narcotics as needed. Topical or oral steroids are also used. Patients with urinary retention may need bladder drainage. Specialist care may also be needed.

Questions for Further Discussion
1. What causes oral ulcers? A review can be found here
2. What causes vaginal discharge? A review can be found here
3. What is in the differential diagnosis of urinary hesitancy? A review can be found here

Related Cases

Disease: Lipschultz Ulcer | Vulvar Disorders | Vaginal Diseases

Symptom/Presentation: Pain | Rash | Urine

Specialty: Adolescent Medicine | Emergency Medicine | Obstetrics / Gynecology

Age: School Ager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Vulvar Disorders and Vaginal Diseases.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Rosman IS, Berk DR, Bayliss SJ, White AJ, Merritt DF. Acute genital ulcers in nonsexually active young girls: case series, review of the literature, and evaluation and management recommendations. Pediatr Dermatol. 2012;29(2):147-153. doi:10.1111/j.1525-1470.2011.01589

Schindler Leal AA, Piccinato CA, Beck APA, Gomes MTV, Podgaec S. Acute genital ulcers: keep Lipschutz ulcer in mind. Arch Gynecol Obstet. 2018;298(5):927-931. doi:10.1007/s00404-018-4866-6

Chen W, Plewig G. Lipschutz genital ulcer revisited: is juvenile gangrenous vasculitis of the scrotum the male counterpart? J Eur Acad Dermatol Venereol. 2019;33(9):1660-1666. doi:10.1111/jdv.15598

Sadoghi B, Stary G, Wolf P, Komericki P. Ulcus vulvae acutum Lipschutz: a systematic literature review and a diagnostic and therapeutic algorithm. J Eur Acad Dermatol Venereol. 2020;34(7):1432-1439. doi:10.1111/jdv.16161

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa