Dear Editor,
A formerly healthy, unmarried 19-year-old woman presented to us with a genital ulcer of three weeks duration. At admission, the patient complained of flu-like symptoms. Despite being initially painless, on further inquiry, the patient reported mild discomfort and dysuria. Her pain score was 3/10 at baseline and 7/10 with urination. There was no history of trauma or sexual contact. Cutaneous examination revealed a single ulcer approximately 5 × 2 cm in dimension over left labia minora with well-demarcated regular margins and pale-yellow slough at the base [Figure 1]. The ulcer was tender on palpation and did not involve the perianal or anal area. There was no inguinal lymphadenopathy, and oral examination did not reveal the presence of aphthae or signs of stomatitis. Pathergy test was negative.
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Figure 1: Ulcer over left labia minora with regular margins at the time of biopsy showing the site of suture (yellow arrow)
Laboratory findings revealed anemia (hemoglobin 10.1 mg/dL) and a leukocyte count of 8600 cells/cumm. Urine microscopy was normal. The culture of vaginal swabs reported moderate growth of candida species. Serological tests for Herpes simplex virus, Human Immunodeficiency Virus, Hepatitis B and C virus, Venereal Disease Research Laboratory (VDRL), Treponema pallidum hemagglutination assay (TPHA) were negative. Gram staining, dark ground microscopy, wound swab culture and Tzanck smear did not reveal any significant findings. However, polymerase chain reaction (PCR) for Epstein-Barr virus (EBV) and Cytomegalovirus (CMV) was not performed. A tissue smear from the genital ulcer showed mature squamous cells dispersed in a background of mixed inflammatory infiltrate with no evidence of Donovan bodies. Histopathological examination of a biopsy taken from the edge of the ulcer showed a non-specific dense dermal infiltrate of neutrophils, lymphocytes, and plasma cells [Figure 2]. Lipschütz ulcer (LU) was subsequently diagnosed following the exclusion of venereal and other non-venereal causes.
Figure 2: Dense dermal infiltration with neutrophils, lymphocytes and plasma cells (H&E 10×)
Treatment was begun with parenteral ceftriaxone, metronidazole and povidone-iodine douching with topical mupirocin ointment empirically. The patient was maintained on oral doxycycline and topical antibiotics at the time of discharge. The patient was followed up for three years with no scarring or recurrence of the ulcer.
LU were first described in 1912 by an Austrian dermatologist Benjamin Lipschütz.[1] They present as acute painful genital ulcers along with systemic symptoms such as fever, malaise, tonsillitis, and lymphadenopathy.
Our patient complained of only mild discomfort at the site of the ulcer and hence the possibility of chancre was ruled out with negative dark ground microscopy, VDRL, and TPHA tests at presentation and during the three year follow-up period. Establishing the diagnosis of LU is by fulfilment of five major diagnostic criteria and one out of two minor diagnostic criteria[2] [Table 1].
Table 1: Diagnostic criteria for Lipschütz ulcer
Infectious diseases such as CMV infection, Mycoplasma infection, influenza, paratyphoid fever, and most commonly EBV[2] infection have often been associated with LU. One proposed mechanism is that of a type III hypersensitivity reaction secondary to viral or bacterial infections forming immunocomplexes that deposit in localized sites, leading to micro thrombosis of dermal vasculature and ultimately causing necrotic ulcers.[3] Skin biopsy usually reveals non-specific mixed inflammatory infiltrate in the dermis and leukocytoclastic vasculitic picture.[2]
LU can present in three morphological variants: (a) Gangrenous type with deep ulcers covered with slough that heal with scarring, (b) Chronic type -which is a recurring form with edema and superficial ulcers, (c) Miliary type– which is fibrinous and more superficial.[4] Our patient had the gangrenous morphological variant.
LU is self-limiting, however, treatment with a short course of systemic corticosteroids (0.5 mg/kg of prednisolone for 1-2 weeks) and topical antibiotics helps in the rapid recovery of ulcers.[5] LU is perplexing to the physician and exasperating to the affected patient. Hence it is of paramount importance for practicing clinicians to be aware of this rare and intriguing condition.
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The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
References
1. Lipschütz B. About a peculiar form of ulcer of the female genitals (Ulcus vulvae acutum). Arch Dermatol Res 1912;114:363–96.
2. Govindan B. Lipschütz ulcers:A literature review based on 79 cases. Repro Health 2016;2:73–8.
3. Moise A, Nervo P, Doyen J, Kridelka F, Maquet J, Vandenbossche G. Ulcer of Lipschutz, a rare and unknown cause of genital ulceration. Facts Views Vis Obgyn 2018;10:55–7.
4. Török L, Domján K, Faragó E. Ulcus vulvae acutum. Cutis 2000;65:387–9.
5. Vismara SA, Lava SA, Kottanattu L, Simonetti GD, Zgraggen L, Clericetti CM, et al. Lipschütz's acute vulvar ulcer:A systematic review. Eur J Pediatr 2020;179:1559–67.
