Lipschutz Ulcer – Nonsexual Acute Genital Ulceration in a 10-year-old Girl : Indian Journal of Paediatric Dermatology

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Case Report

Lipschutz Ulcer – Nonsexual Acute Genital Ulceration in a 10-year-old Girl

Radhika, S. R.; Shilpa, Kanathur; Shree Lakshmi, H. V.; Revathi, T. N.

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Indian Journal of Paediatric Dermatology 22(2):p 154-156, Apr–Jun 2021. | DOI: 10.4103/ijpd.IJPD_80_20
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Abstract

Introduction

The cause of genital ulcers is mostly infectious. Other causes include autoimmune conditions such as Behcet's disease, trauma, drug reactions, or other inflammatory processes. Lipschutz ulcer (LU) is a nonsexually transmitted cause of acute genital ulcer commonly seen in young women. However, there are a few reported cases in children. A recent study has reported that LU represents nearly 30% of vulvar ulcerations.[1] It clinically presents with an acute onset of painful vulvar ulcers, which are more than 1 cm wide and deep. They are surrounded by a red border with a necrotic center, which is covered by grey exudate. It has been observed that it may be preceded by nonspecific symptoms like flu-like syndrome. The characteristic lesions are bilateral “kissing lesions.” The diagnosis is mainly by clinical examination and is made by excluding other usual causes of genital ulceration. Although the etiology is not known, recent reports have associated it with infections such as Epstein–Barr virus (EBV), cytomegalovirus (CMV), Mycoplasma pneumoniae, Mycoplasma fermentans, mumps, Parvovirus B19, coinfection of influenza B and adenovirus, and coinfection of EBV and CMV.[2]

Case Report

A 10-year-old girl came with complaints of a painful wound over the genitals since 4 days. There was a history of preceding fever, sore throat, and mild cough of 5 days’ duration. There was no history of sexual exposure, drug intake, or trauma to the site prior to the onset of the lesion. She did not complain of any oral lesions or joint pains. The patient did not have a history of similar lesions. Genital examination revealed a well-defined large polycyclic ulcer of size 5 cm × 3 cm with red granulation tissue on the floor located over the left labia majora extending into the vagina and clitoris. Two small superficial ulcers coalesced with the main ulcer and a small ulcer of size 0.5 cm in diameter was present to the left of the main ulcer. A superficial ulcer of size 1 cm in diameter was present on the inner aspect of the right labia majora extending into the vagina, thus giving a kissing ulcer appearance [Figure 1]. Purulent discharge was present over the inner aspect of the left labia majora. On palpation, the ulcer was non indurated, tender, and did not bleed on touch. Lymph nodes were nonpalpable. No other abnormality was detected on mucosal, cutaneous, and systemic examination. Thus, a differential diagnosis of herpes genitalis, chancroid, LU, and Behcet's disease was made. Gram stain, pus culture, Tzanck smear, HIV 1 and 2, Venereal Disease Research Laboratory (VDRL), and HSV 1 and 2 immunoglobulin M (IgM) titers were negative. EBV IgM titers were also negative. Biopsy was not done as the patient's mother did not consent. Pathergy test was negative and no history suggestive of Behcet's or Crohn's disease was present. With the above clinical findings and investigations, a final diagnosis of LU was made. The patient was treated with topical mupirocin ointment for 4 weeks and oral anti-inflammatory drugs for 5 days [Figure 2]. The child showed complete clearance of the lesions in a month with only a residual scar [Figure 3]. The patient was followed up for 10 months and no recurrence was noted.

F1-12
Figure 1:
Large ulcers with purulent exudate giving it a “kissing ulcer” like appearance
F2-12
Figure 2:
Lesions healing 15 days after treatment with topical mupirocin ointment
F3-12
Figure 3:
Complete healing of ulcers with residual scar

Discussion

LU, an acute ulcer of the vulva, is a rare entity, initially described by Lipschutz in 1913.[3] As with our case, it is usually seen in young virgin girls before the beginning of their sexual life. The exact etiology is unknown, but it is thought to be a contemporary episode of infection. Although multiple hypotheses have been put forward, the pathogenesis of LU remains a mystery. It has been said that LU could develop from either hematogenous spread or autoinoculation. According to one of the hypotheses, it is a hypersensitivity reaction to a viral or bacterial infection, leading to the deposition of immune complexes in the dermal vessels. This, in turn, activates the complement system, resulting in the formation of a microthrombus and subsequent tissue necrosis.[45] It could also be due to cytolysis of the tissue, as seen in HSV infections.[6] The initial symptoms can be flu-like, such as malaise, fever, tonsillitis, and lymphadenopathy. Later on, it manifests as a single or multiple deep and painful ulcers on the vulva. LU can present with different morphological patterns, such as pseudovesicles, herpetiform lesions, or ulcers with an eschar.[7] Sometimes, patients may also develop morbilliform-like rashes or erythema nodosum-like lesions. Torok et al.[8] described three other forms of LU-gangrenous, chronic, and miliary (apart from the classical clinical ulcerative presentation). The clinical workup starts with a detailed sexual history and a history concerning symptoms of other inflammatory disorders such as Behcet's disease and Crohn's disease. A thorough cutaneous, mucosal, ocular, and systemic examination needs to be carried out. Laboratory investigations suggested are complete blood count, STD panel, EBV IgM titers, and a skin biopsy from the ulcer edge for ulcers lasting longer than 3 or 4 weeks. Histology shows a lymphocytic arteritis to leukocytoclastic vasculitis picture, which has limited diagnostic value.[9] Our patient completely fulfilled the diagnostic criteria for LU [Table 1], having all five major and both minor criteria. Thus, a diagnosis of LU was made in our case after ruling out all other causes of acute genital ulceration. LU treatment mainly aims at pain relief and ulcer healing. Although LU is a self-limiting condition, a brief course of systemic corticosteroids (0.5 mg/kg of prednisolone for 1–2 weeks) may be required for large and deep ulcers.[10] Antivirals do not have an important role in LU treatment. Follow-up is to be weekly until the ulcer has healed, followed by yearly to rule out the progression to systemic inflammatory diseases.

T1-12
Table 1:
Diagnostic criteria for Lipschutz ulcer[2]

Conclusion

We report a rare case of LU occurring in a 10-year-old girl. Dermatologists must be aware of Lipshutz ulcer as a cause of acute genital ulcer.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published, and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

1. Vieira-Baptista P, Lima-Silva J, Beires J, Martinez-de-Oliveira J. Lipschütz ulcers: Should we rethink this? An analysis of 33 cases Eur J Obstet Gynecol Reprod Biol. 2016;198:149–52
2. Govindan B. Lipschütz ulcers: A literature review based on 79 cases EMJ Reprod Health. 2016;2:73–8
3. Lipschutz B. A peculiar ulcer of the female genital (ulcus vulvae acutum) Arch Dermatol Syph. 1913;114:363–95
4. Leigh R, Nyirjesy P. Genitourinary manifestations of Epstein-Barr virus infections Curr Infect Dis Rep. 2009;11:449–56
5. Portnoy J, Ahronheim GA, Ghibu F, Clecner B, Joncas JH. Recovery of Epstein-Barr virus from genital ulcers N Engl J Med. 1984;311:966–8
6. Sixbey JW, Nedrud JG, Raab-Traub N, Hanes RA, Pagano JS. Epstein-Barr virus replication in oropharyngeal epithelial cells N Engl J Med. 1984;310:1225–30
7. Huppert JS. Lipschutz ulcers: Evaluation and management of acute genital ulcers in women Dermatol Ther. 2010;23:533–40
8. Török L, Domján K, Faragó E. Ulcus vulvae acutum Cutis. 2000;65:387–9
9. Covino JM, McCormack WM. Vulvar ulcer of unknown etiology in a human immunodeficiency virus-infected woman: Response to treatment with zidovudine Am J Obstet Gynecol. 1990;163:116–8
10. Lampert A, Assier-Bonnet H, Chevallier B, Clerici T, Saiag P. Lipschutz's genital ulceration: A manifestation of Epstein-Barr virus primary infection Br J Dermatol. 1996;135:663–5
Keywords:

Genital ulcer; Lipschutz ulcer; nonvenereal cause; ulcus vulvae acutum

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