Lipschütz ulcers: should we rethink this? An analysis of 33 cases

Abstract

Objectives

To describe the presentation and characteristics of patients with Lipschütz ulcers (LU) and to evaluate the role of a standard protocol in the aetiological diagnosis.

Study design

Retrospective analysis of all cases of diagnosis of LU at our Vulvar Clinic during a five-year period.

Results

Of 110 women with vulvar ulcers, 33 (30.0%) had a diagnosis of LU. The mean age was 29.1 ± 15.14 years (10–79 years). Nine (27.3%) were 35 years old or more. The majority had had their sexual debut (28, 84.8%). Ten patients (30.3%) referred had at least one previous similar episode. Twenty-five (75.7%) had non-gynecological symptoms in the week before. The ulcers were located most frequently on the vestibule (19, 57.6%) and the labia minora (10, 30.3%). Isolated lesions on the left side were uncommon (3, 9.1%). Most had multiple (22, 66.7%) lesions. The mean time to full healing of the lesions was 15.6 ± 6.20 days.

A microbiological possible cause was identified in 9 (27.3%) patients: CMV (3 cases), Mycoplasma pneumoniae (3 cases), EBV (2 cases) and PVB19 (1 case). The protocol did not include systematic biopsies, blood count differentials, C-reactive protein and liver enzymes, which may have lead to some missed diagnosis.

Conclusions

LU can be found in women of any age, most of them sexually active. In most cases the lesions are preceded by non-gynecological symptoms and recurrence is common. Most lesions occur on the vestibule and labia minora, being rarely found isolated on the left side. Viruses seem to be the most frequent associated agents but Mycoplasma pneumoniae serology should be considered. In one case there was a possible role for PVB19. A standard protocol can effectively exclude sexually transmitted diseases and lead to a diagnosis in up to a third of cases.

Introduction

Lipschütz ulcers (LU) (also known as ulcus vulvae acutum, acute vulvar ulcers, primary aphthous ulcers or reactive non-sexually related acute genital ulcers) (Fig. 1, Fig. 2) were first described in 1913 [1]. These ulcers are considered to have a sudden onset, to be acute, painful and nonsexually transmitted. Systemic symptoms, such as fever, myalgias, headache, diarrhea, oral aphthae, enlarged lymph nodes, tonsillitis or respiratory symptoms can be present. Healing occurs spontaneously, sometimes with scarring; the chance of recurrence is questionable. It is considered to be more frequent in girls or young women, who frequently are virgins. The diagnosis is one of exclusion, after ruling out sexually transmitted infections, idiopathic aphthosis, Behçet's syndrome, extra-genital Crohn's disease[2] or any other specific diagnosis.

LU are frequently undiagnosed and thus its true incidence is unknown. However, it can be a great cause of anxiety to patients and their parents (when in children) as well as to doctors, to whom it is sometimes an unknown entity [3].

In recent years, these ulcers have been related to infectious agents, including Epstein–Barr virus (EBV) [4], Mycoplasma pneumoniae [5] cytomegalovirus (CMV) [6] and Salmonella [7], among others. The mechanisms that lead to the formation of ulcers distant to the site of the primary infection are poorly understood.

It is useful to relate these lesions to a non-sexually transmitted agent, as it can help to relief some anxiety. Knowing that there is a specific agent related to the lesions can be helpful also to avoid an over-diagnosis of Behçet's syndrome, if other characteristics of the latter are also present.

There are no large series of cases of LU published. Most of the data derives from sporadic or anecdotal cases, and a significant number of those come from the pediatric literature.

The authors intend to describe the characteristics of the affected patients, the form of presentation of the condition and to evaluate up to what point a standard protocol is useful in terms of reaching an aetiological diagnosis.