Ulcus vulvae acutum Lipschütz in two young female patients

Abstract

Ulcus vulvae acutum Lipschütz in two young female patientsUlcus vulvae acutum Lipschütz or acute genital ulcer is a distinct clinical entity characterized by sudden painful genital ulceration occurring mostly in young and virgin girls with malaise, fever and other systemic symptoms. This distressing syndrome is rare and may be presented to dermatologists, gynecologists or pediatricians. Its diagnosis and therapy can be challenging. We present two young female patients with ulcus vulvae acutum. The cause of the disease could not be confirmed in our patients, but, interestingly, both patients had partial IgA deficiency. In the last 100 years, after its first description by Lipschütz, many case reports and series have aimed to identify a specific cause of the disease, without success. These studies mainly focused on infectious agents as causative factors, however, in most cases connection with infection could not be confirmed. Our opinion is that the decreased level of IgA could be a possible explanation for the cause of this syndrome. With our cases we would like to emphasize the possible role of local immunological mechanisms rather than several infectious agents in the development of this little-known disease.

Ulcus vulvae acutum — A case of genital ulcers in adolescent girl

“…Numerous etiologies have been proposed such as Mycoplasma infection, paratyphoid fever, influenza A infection, cytomegalovirus (CMV) associated acute mononucleosis and mostly EBV infection, as in the present case, but pathogenesis is still unknown [1] . If the hypothesis of an interplay between a direct cytolytic effect of EBV replication in the vulvar epithelium and the associated inflammatory reaction is true, whether the infectious agent reaches the genital mucosa directly, via hematogenous transport by circulating infected T lymphocytes or through autoinoculation of oral secretion it is similarly debated [5] , [8] . Moreover Farhi et al [9] found negative results of in situ hybridization for EBV in three of four samples studied suggesting that ulcus vulvae acutum may be more likely to result from an indirect immune reaction than from a direct epithelial cytopathogenic effect of EBV [10] .…”

“…In fact, the systemic infective illness leads the recruitment of cytotoxic T-lymphocytes, found in the infiltrate, that are responsible of marked inflammation and consequently genital ulceration [5] , [10] . Moreover, a new recent report has emphasized the possible role of local immunological mechanisms in the development of the disease presenting two cases of ulcus vulvae acutum in young females with partial IgA deficiency [8] . They supposed that, in order to compensate the decreased levels of IgA, it will produce a pronounced T-helper 1 reaction, resulting in a stronger local cytotoxic immune response of the mucosa.…”

Lipschütz genital ulcer revisited: is juvenile gangrenous vasculitis of the scrotum the male counterpart?

“…Based on retrospective hospital‐based studies of women with acute genital ulcers, it is reported to vary at 4–35%, depending on the diagnostic criteria used, given that no consensus actually exists for the disease diagnosis. Most of the subsequent reports focus on the first type of ulcer described by Lipschütz displaying deep ulceration with excruciating pain of acute febrile onset, which rarely recurs . Only a few reports described the afebrile, painless and recurrent nature of the second type of ulcer which was clearly pointed out by Lipschütz .…”

“…Most of the subsequent reports focus on the first type of ulcer described by Lipsch€ utz displaying deep ulceration with excruciating pain of acute febrile onset, which rarely recurs. [6][7][8][9] Only a few reports described the afebrile, painless and recurrent nature of the second type of ulcer which was clearly pointed out by Lipsch€ utz. 10,11 Some retrospective studies no longer distinguished between the two kinds of ulcers mentioned by Lipsch€ utz.…”

Acute Genital Ulcer in a Young Girl

“…Lipschütz ulcer or ulcus vulvae acutum is an uncommon and probably underdiagnosed entity characterized by an acute, painful vulvar ulceration of non-venereal origin. [1][2][3][4][5][6] It is considered to be more common in adolescents or young women, who frequently are virgins, but several cases have also been reported in children. [1][2][3][4][5][6] The etiology remains unknown.…”