Vulvar Aphthous Ulcer

Author:
Alyson J. Brinker, MD
US Naval Hospital Guam, Agana Heights, Guam

Citation:
Brinker AJ. Vulvar aphthous ulcer. Consultant. 2017;57(7):445-446.

A 29-year-old woman presented with a quarter-sized and exquisitely painful ulceration on the vulva near the introitus. The lesion had begun as a superficial violaceous patch 5 days prior, and it had worsened, forming a whitish pseudomembrane which she had accidentally removed when wiping. She reported that the ulcer was associated with some of the worst pain she had ever had, especially with urination. She had been self-treating with hydrocortisone and Vagisil for pain control. She had also concurrently developed several oral aphthous ulcers.

History. Her most recent sexual encounter had been with a woman 6 weeks previously. She denied a history of a diagnosed sexually transmitted infection (STI); however, she did state that she “might have undiagnosed herpes,” since she had experienced smaller outbreaks of genital ulcers and dysuria about once a year for the past 5 years. These ulcers had always resolved spontaneously, and the lesions had always been smaller than 2 to 3 mm. She denied vaginal discharge or abnormal odor. She denied recent illnesses or enlarged lymph nodes. She did mention having experienced subjective mild fever and chills the previous night or two. She denied other symptoms or other sexual contacts.

Physical examination. At presentation, examination of the vulva revealed a clean-based ulcer measuring 1.1 × 2.3 cm, approximately 0.25 cm in depth, with sharply demarcated borders and an erythematous rolled edge (Figure 1).

Pending laboratory test results, the patient initially was treated with oral trimethoprim-sulfamethoxazole presumptively for possible methicillin-resistant Staphylococcus aureus infection. The patient noted no improvement over the course of 7 days, however. Results of a full workup for STIs were negative, including negative immunoglobulin G and M results for herpes simplex virus (HSV). The wound culture results were normal. Test results for Epstein-Barr virus (EBV) were negative. No biopsy was done at the patient’s request. The pain was treated adequately with topical lidocaine, 1%, and slowly (over the course of 40 days), the lesion healed (Figure 2). The attending obstetrician-gynecologist did not prescribe a topical corticosteroid cream.

Discussion. Acute genital aphthous ulceration is an uncommon, nonsexually transmitted condition that is characterized by the rapid onset of painful, necrotic ulcerations of the vulva or lower vagina. The lesions are thought to be similar in pathology, appearance, and symptomatology to the much more familiar and common oral canker sore.¹ The classic description is sharply marginated and “punched out” in appearance, with a rolled erythematous border. Such ulcers can, as in this patient’s case, have a white coagulum initially, and can be large (up to 3 cm) in comparison with oral ulcers.

Other remote considerations include Crohn disease (associated vulvar lesions are usually “knife-cut” in appearance), HSV (in immunocompromised patients), trauma, malignancy, pyoderma gangrenosum, bullous disorders, erythema multiforme, and erosive lichen planus. Vulvar aphthosis has been associated with EBV,^2,3 cytomegalovirus,⁴ influenza A virus,⁵ mumps,⁶ paratyphoid,⁷ Mycoplasma pneumoniae,⁸ and Lyme disease.⁹

Complex aphthosis is a term that describes patients with recurrent oral and genital aphthae in the absence of other clinical features of Behçet syndrome.¹⁰ Given her history of both oral ulcers and smaller vulvar ulcers, this patient likely fits this diagnosis. Behçet syndrome is an often overdiagnosed, immune-mediated, small-vessel systemic vasculitis with associated ophthalmologic problems and systemic symptoms involving visceral organs, including the gastrointestinal tract and the pulmonary, musculoskeletal, cardiovascular, and neurologic systems. There is no diagnostic test for Behçet syndrome, except for agreed-upon clinical criteria including at least 2 of the following: recurrent genital ulcers, ocular inflammation, defined skin lesions, and pathergy for Behçet syndrome. A diagnosis of complex aphthosis can be made for any patient with recurrent oral and genital aphthae without systemic manifestations.

Acute genital ulceration secondary to autoimmune processes typically heals spontaneously, and treatment is primarily supportive and includes reassurance, local hygiene, wound care, and pain control with nonsteroidal anti-inflammatory drugs and topical anesthetics.^11,12 Patients should be reassured that the ulcers are not infectious nor sexually transmitted, and that recurrence is infrequent. A course of topical corticosteroids, even up to class 2 or class 1, can be trialed for a brief period and would have been a reasonable approach in this patient’s case.

The differential diagnosis of vulvar ulceration is broad and should include an appropriate workup to rule out acute infectious etiologies. Presumptive treatment with antibiotics is typical but unnecessary if no risk factors are present. Most important are pain control, reassurance, and long-term follow-up (often a significantly extended time) to monitor for recurrences, which can lead to several other and more severe disease processes on the continuous spectrum of recurrent urogenital aphthosis.

REFERENCES:

1. Lehman JS, Bruce AJ, Wetter DA, Ferguson SB, Rogers RS III. Reactive nonsexually related acute genital ulcers: review of cases evaluated at Mayo Clinic. J Am Acad Dermatol. 2010;63(1):44-51.
2. Jerdan K, Aronson I, Hernandez C, Fishman PM, Groth JV. Genital ulcers associated with Epstein-Barr virus. Cutis. 2013;91(6):273-276.
3. Sárdy M, Wollenberg A, Niedermeier A, Flaig MJ. Genital ulcers associated with Epstein-Barr virus infection (ulcus vulvae acutum). Acta Derm Venereol. 2011;91(1):55-59.
4. Martín JM, Godoy R, Calduch L, Villalon G, Jordá E. Lipschütz acute vulval ulcers associated with primary cytomegalovirus infection. Pediatr Dermatol. 2008;25(1):113-115.
5. Wetter DA, Bruce AJ, MacLaughlin KL, Rogers RS III. Ulcus vulvae acutum in a 13-year-old girl after influenza A infection. Skinmed. 2008;7(2):95-98.
6. Chanal J, Carlotti A, Laude H, Wallet-Faber N, Avril M-F, Dupin N. Lipschütz genital ulceration associated with mumps. Dermatology. 2010;221(4):292-295.
7. Pelletier F, Aubin F, Puzenat E, et al. Lipschütz genital ulceration: a rare manifestation of paratyphoid fever. Eur J Dermatol. 2003;13(3):297-298.
8. Vieira-Baptista P, Machado L, Costa AR, Beires J, Martinez-de-Oliveira J. Mycoplasma pneumoniae: a rare cause of vulvar ulcers or an undiagnosed one? J Low Genit Tract Dis. 2013;17(3):330-334.
9. Finch JJ, Wald J, Ferenczi K, Khalid S, Murphy M. Disseminated Lyme disease presenting with nonsexual acute genital ulcers. JAMA Dermatol. 2014;​150(11):1202-1204.
10. Rogers RS III. Complex aphthosis. Adv Exp Med Biol. 2003;528:311-316.
11. Huppert JS. Lipschutz ulcers: evaluation and management of acute genital ulcers in women. Dermatol Ther. 2010;23(5):533-540.
12. Bandow GD. Diagnosis and management of vulvar ulcers. Dermatol Clin. 2010;28(4):753-763.