Acute genital ulceration (Lipschütz ulcer)
- Robert Sidbury, MD, MPH
Robert Sidbury, MD, MPH
- Professor - Department of Pediatrics
- University of Washington School of Medicine
Acute genital ulceration, also known as Lipschütz ulcer or "ulcus vulvae acutum," is an uncommon, nonsexually transmitted condition characterized by the rapid onset of painful necrotic ulcerations of the vulva or lower vagina. It typically occurs in sexually inactive adolescent girls or young women and may be preceded by influenza-like or mononucleosis-like symptoms. Acute genital ulceration has been associated with acute Epstein-Barr virus (EBV) infection or other viral and bacterial infections [1-4]. However, in many cases a cause cannot be determined [5-7].
This topic will review the clinical manifestations, diagnosis, and management of acute genital ulceration. Other causes of vulvar ulceration are discussed separately. (See "Vulvar lesions: Differential diagnosis based on morphology", section on 'Ulcers' and "Approach to the patient with genital ulcers".)
The precise incidence of acute genital ulceration is unknown. In a few cases series, the average age at onset was 12 to 29 years [1,4,8]. There are rare reports of acute genital ulcerations in adult women and young children [9,10].
ETIOLOGY AND PATHOGENESIS
Several lines of evidence suggest that acute genital ulceration may be a manifestation of primary infection with Epstein-Barr virus (EBV) [1,5,11-13]. EBV might reach the genital mucosa via hematogenous spread of EBV-infected lymphocytes or Langerhans cell precursors or through autoinoculation with saliva, urine, or cervicovaginal fluid . (See "Clinical manifestations and treatment of Epstein-Barr virus infection", section on 'Primary infection'.)
There are isolated reports of acute genital ulceration associated with infection with other viruses and bacteria, including cytomegalovirus , influenza A virus , influenza B virus , mumps virus , salmonella , and mycoplasma , and with disseminated Lyme disease . In one report, genital ulcers were the first manifestation of acute myeloid leukemia in a 28-year-old woman . However, in most cases a cause cannot be determined [5-7].
- Farhi D, Wendling J, Molinari E, et al. Non-sexually related acute genital ulcers in 13 pubertal girls: a clinical and microbiological study. Arch Dermatol 2009; 145:38.
- Di Lernia V, Mansouri Y. Epstein-Barr virus and skin manifestations in childhood. Int J Dermatol 2013; 52:1177.
- Lehman JS, Bruce AJ, Wetter DA, et al. Reactive nonsexually related acute genital ulcers: review of cases evaluated at Mayo Clinic. J Am Acad Dermatol 2010; 63:44.
- Jerdan K, Aronson I, Hernandez C, et al. Genital ulcers associated with Epstein-Barr virus. Cutis 2013; 91:273.
- Hernández-Núñez A, Córdoba S, Romero-Maté A, et al. Lipschütz [corrected] ulcers--four cases. Pediatr Dermatol 2008; 25:364.
- Cebesoy FB, Balat O, Inaloz S. Premenarchal vulvar ulceration: is chronic irritation a causative factor? Pediatr Dermatol 2009; 26:514.
- Deitch HR, Huppert J, Adams Hillard PJ. Unusual vulvar ulcerations in young adolescent females. J Pediatr Adolesc Gynecol 2004; 17:13.
- 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.
- Brinca A, Canelas MM, Carvalho MJ, et al. Lipschütz ulcer (ulcus vulvae acutum): a rare cause of genital lesion. An Bras Dermatol 2012; 87:622.
- Burguete Archel E, Ruiz Goikoetxea M, Recari Elizalde E, et al. Lipschütz ulcer in a 17-month-old girl: a rare manifestation of Epstein-Barr primoinfection. Eur J Pediatr 2013; 172:1121.
- Halvorsen JA, Brevig T, Aas T, et al. Genital ulcers as initial manifestation of Epstein-Barr virus infection: two new cases and a review of the literature. Acta Derm Venereol 2006; 86:439.
- Taylor S, Drake SM, Dedicoat M, Wood MJ. Genital ulcers associated with acute Epstein-Barr virus infection. Sex Transm Infect 1998; 74:296.
- Sárdy M, Wollenberg A, Niedermeier A, Flaig MJ. Genital ulcers associated with Epstein-Barr virus infection (ulcus vulvae acutum). Acta Derm Venereol 2011; 91:55.
- Martín JM, Godoy R, Calduch L, et al. Lipschütz acute vulval ulcers associated with primary cytomegalovirus infection. Pediatr Dermatol 2008; 25:113.
- Wetter DA, Bruce AJ, MacLaughlin KL, Rogers RS 3rd. Ulcus vulvae acutum in a 13-year-old girl after influenza A infection. Skinmed 2008; 7:95.
- Haidari G, MacMahon E, Tong CY, White JA. Genital ulcers: it is not always simplex …. Int J STD AIDS 2015; 26:72.
- Chanal J, Carlotti A, Laude H, et al. Lipschütz genital ulceration associated with mumps. Dermatology 2010; 221:292.
- Pelletier F, Aubin F, Puzenat E, et al. Lipschütz genital ulceration: a rare manifestation of paratyphoid fever. Eur J Dermatol 2003; 13:297.
- Vieira-Baptista P, Machado L, Costa AR, et al. Mycoplasma pneumoniae: a rare cause of vulvar ulcers or an undiagnosed one? J Low Genit Tract Dis 2013; 17:330.
- Finch JJ, Wald J, Ferenczi K, et al. Disseminated Lyme disease presenting with nonsexual acute genital ulcers. JAMA Dermatol 2014; 150:1202.
- Gawai SR, Binesh VG, Betsy A, Jisha KT. An unusual initial manifestation of acute myeloid leukemia. Int J STD AIDS 2017; 28:418.
- Huppert JS. Lipschutz ulcers: evaluation and management of acute genital ulcers in women. Dermatol Ther 2010; 23:533.
- Barrett MM, Sangüeza M, Werner B, et al. Lymphocytic Arteritis in Epstein-Barr Virus Vulvar Ulceration (Lipschütz Disease): A Report of 7 Cases. Am J Dermatopathol 2015; 37:691.
- Letsinger JA, McCarty MA, Jorizzo JL. Complex aphthosis: a large case series with evaluation algorithm and therapeutic ladder from topicals to thalidomide. J Am Acad Dermatol 2005; 52:500.
- Rogers RS 3rd. Complex aphthosis. Adv Exp Med Biol 2003; 528:311.
- Farrell AM, Kirtschig G, Dalziel KL, et al. Childhood vulval pemphigoid: a clinical and immunopathological study of five patients. Br J Dermatol 1999; 140:308.
- Lebeau S, Mainetti C, Masouyé I, et al. Localized childhood vulval pemphigoid treated with tacrolimus ointment. Dermatology 2004; 208:273.
- Guenther LC, Shum D. Localized childhood vulvar pemphigoid. J Am Acad Dermatol 1990; 22:762.
- Hoque SR, Patel M, Farrell AM. Childhood cicatricial pemphigoid confined to the vulva. Clin Exp Dermatol 2006; 31:63.
- Bandow GD. Diagnosis and management of vulvar ulcers. Dermatol Clin 2010; 28:753.
- Rosman IS, Berk DR, Bayliss SJ, et al. Acute genital ulcers in nonsexually active young girls: case series, review of the literature, and evaluation and management recommendations. Pediatr Dermatol 2012; 29:147.
- Dixit S, Bradford J, Fischer G. Management of nonsexually acquired genital ulceration using oral and topical corticosteroids followed by doxycycline prophylaxis. J Am Acad Dermatol 2013; 68:797.
- Liu C, Zhou Z, Liu G, et al. Efficacy and safety of dexamethasone ointment on recurrent aphthous ulceration. Am J Med 2012; 125:292.
- Femiano F, Buonaiuto C, Gombos F, et al. Pilot study on recurrent aphthous stomatitis (RAS): a randomized placebo-controlled trial for the comparative therapeutic effects of systemic prednisone and systemic montelukast in subjects unresponsive to topical therapy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010; 109:402.
- Huppert JS, Gerber MA, Deitch HR, et al. Vulvar ulcers in young females: a manifestation of aphthosis. J Pediatr Adolesc Gynecol 2006; 19:195.