Case presentation: Acute genital ulceration in a 15-year-old girl

A previously healthy 15-year-old female presented to the emergency department with 6 days of daily fevers, and 72 hours of non-bloody diarrhea that had self-resolved. In addition, she complained of nausea and a headache. Notably, in the last day, she had developed labial swelling, perianal pruritus, and vaginal burning.

On history, her last menstrual period was 1 week ago, and she was not sexually active.

It was noted that she had consumed Kinder-Surprise eggs in the last week, which had been recently recalled for Salmonella contamination.

Pelvic exam initially showed multiple hemorrhagic vesicles involving the labia minora (Figure 1A). On day one of admission, she developed two symmetric, well-delimited ulcers at the 5 o’clock and 7 o’clock positions on the labia minora, each 12 mm × 5 mm in size (Figure 1B). The remainder of her physical examination was normal.

Figure 1.

(A) Hemorrhagic vesicles and edema of the labia minora. (B) Large necrotic, sharply demarcated ulcer, with smaller ulcer inferiorly, on the right labia minora.

Laboratory investigations revealed a C-reactive protein (CRP) of 16.4 mg/L (normal ≤ 10.0 mg/L). Urinalysis was 2+ positive for blood, and 3+ positive for protein. The platelet and coagulation profiles were normal. Blood cultures were positive for Gram-negative bacilli. By the second day of admission, she had dysuria requiring catheterizing. Further diagnostic tests revealed the cause of her painful vulvar ulcers.

CASE DIAGNOSIS: LIPSCHÜTZ’S ULCERS AND NON-TYPHOID Salmonella BACTEREMIA

Discussion

The differential diagnosis of genital ulcer disease may be divided into infectious and non- infectious etiologies. The most common causes of genital ulceration are herpes simplex virus (HSV) types 1 and 2 and syphilis (Treponema pallidum) (1). Genital HSV may be asymptomatic or painful, may be preceded by prodromal symptoms of tingling or shooting pain in the buttocks, legs, or hips, and may present with inguinal lymphadenopathy, and fever (1). Primary syphilis may escape recognition as it presents with a single, painless, well-demarcated ‘chancre’ that heals spontaneously in 3 to 6 weeks (1).

Genital ulceration secondary to lymphogranuloma venerum (Chlamydia trachomatis) serotypes L1, L2, and L3 is primarily endemic to tropical and subtropical climates (1). Primary lymphogranuloma venerum infection manifests as a painless genital or rectal papule that ulcerates and heals spontaneously (1). In the secondary stage of infection, there can be unilateral, tender inguinal or femoral lymphadenopathy, and anorectal symptoms, such as rectal bleeding and tenesmus (1). Chancroid (Haemophilus ducreyi) is a common cause of genital ulcer disease in Asia, Africa, and the Caribbean (1). Microabrasions that occur during sexual intercourse result in painful ulcers with soft irregular margins, which may be ‘kissing’ if there are multiple areas of micro trauma or contact between two adjacent areas (1). In half of patients, there is also painful, unilateral inguinal adenitis, which may develop into fluctuant buboes (1).

Non-infectious etiologies of genital ulceration can include cutaneous drug reactions, and mechanical, thermal, or chemical trauma (1). Granulomatous genital inflammation is a rare extra-intestinal manifestation of Crohn’s disease characterized by labial edema, and pathognomonic ‘knife-cut’ vulvar ulcers (1). Behçet’s syndrome, most prevalent in the Middle East and Mediterranean, presents with deep, painful genital ulcers that heal with scarring, alongside aphthous oral ulcers, and intermittent arthritis (1).

In this patient, HSV, chlamydia and gonorrhea were not detected on vaginal swabs, and serologic testing for syphilis was negative. Salmonella montevido was detected on her blood cultures and she was started on intravenous antibiotics. Her vulvar ulcers improved with a conservative regimen of local hygiene, wound care, and pain control. The final diagnosis was of Lipschütz’s ulceration (LU).

LU is an uncommon disease characterized by the sudden onset of necrotic and painful genital ulcers, and typically occurs in young (≤ 20 years old), sexually inactive females who were previously healthy, have no history of genital ulcers, and were not previously affected by a chronic inflammatory condition (2).

In LU, there are usually one to three lesions that are large (≥10 mm), shallow, well-delimited, and have a fibrinous and necrotic centre (2). These tend to occur on the medial aspects of the labia minora and vestibule and have a symmetric ‘kissing’ distribution on opposing surfaces (2).

Voiding disorders, aphthous ulcers, and enlarged inguinal lymph nodes have also been reported in LU (2).

The pathophysiology of LU is not well understood. It often occurs concomitantly with an infectious disease and develops before its resolution. LU is most associated with Epstein-Barr virus, detected in 27% of 139 cases of LU, but has also been described with cytomegalovirus, mycoplasma species, Toxoplasma gondii, influenza A and B (2). To date, there is one other reported case of LU associated with salmonella (2).

LU is self-limited and generally resolves without recurrence or long-term sequelae within 3 weeks (2). For analgesia, topical lidocaine is recommended, especially 5 to 10 minutes before micturition, alongside naproxen and acetaminophen as needed (2). Sitz baths, application of a bland barrier cream before and after voiding, avoidance of pads and underwear, and maintenance of good hydration to minimize irritation by concentrated urine also constitute important components of supportive care for LU (2).

CLINICAL PEARLS

• Lipschütz’s ulceration is a diagnosis of exclusion, which can only be made after an extensive review of systems, sensitive sexual history, examination, and thorough workup for both sexually transmitted infections and non-sexually transmitted diseases causing acute genital ulceration.

• Lipschütz’s ulcers are poorly understood and therefore under-diagnosed, or mis- diagnosed as herpes simplex infection, in which case they are treated with acyclovir without benefit. Identification of an etiologic trigger, such as a viral or bacterial infection, can aid in their appropriate detection.

• Lipschütz’s ulcers resolve spontaneously with supportive care. Patients should receive follow-up or anticipatory guidance to rule out progression to systemic illnesses causing genital ulcers, such as Crohn’s disease or Behçet’s syndrome.

FUNDING

There are no funders to report.

INFORMED CONSENT

Informed consent was obtained for all photographs and patient information detailed.

POTENTIAL CONFLICTS OF INTEREST

All authors: No reported conflicts of interest. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed. There are no conflicts of interest to disclose for any of the authors. There is no funding to disclose. Informed consent was obtained for all photographs and patient details included.