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Description: In general, vulvar ulcers are deep defects through the epidermis into the dermis that generally heal with scarring; they are deeper than erosions. They may be of infectious or noninfectious origin. Acute aphthous ulcers (acute genital ulceration, Lipschütz ulcers [named for Benjamin Lipschütz, who first described it in October, 1912], non-sexually acquired genital ulceration [NSAGU], and vulvar aphthae) are like the more common oral ulcers known as canker sores. These ulcers are the result of a localized vasculitis caused, in about one-third of cases, by various infections, most commonly Epstein-Barr virus, Mycoplasma pneumoniae, and viral respiratory infections (parvovirus, influenza, paramyxovirus). Other infections include tuberculosis, amebiasis, and leishmaniasis. There have even been reports of cases after COVID-19 infection and vaccination.
Prevalence: Uncommon.
Predominant Age: Most common in adolescent girls and young women, typically ages 8–25 years.
Genetics: No genetic pattern. A family history of autoimmune disorders may be present.
Causes: Chronic or recurrent aphthous ulcers are often idiopathic.
Risk Factors: Viral infection.
Exquisitely painful ulcers appear quickly, evolving from a purpuric area that rapidly becomes necrotic, then ulcerating over 1–2 days.
Normally shallow ulcers begin as pale yellow in color, generally turning gray as the condition develops.
Flulike symptoms (fever, malaise, tonsillitis, lymphadenopathy) precede or accompany the ulcers.
Multiple, bilateral ulcers are common.
Noninfectious etiologies —Noninfectious etiologies of genital ulcers: Fixed drug reactions (Stevens-Johnson syndrome, toxic epidermal necrolysis), Behçet syndrome, neoplasms, Crohn disease, malignancy, trauma, vasculitis (lupus), hidradenitis suppurativa, pyoderma gangrenosum, chemical exposure (topical 20% benzocaine), “neurotic excoriations” or self-inflicted injuries.
Non-sexually transmitted infections —Tuberculosis, amebiasis, schistosomiasis, leishmaniasis.
Sexually transmitted infections —Herpes, granuloma inguinale, syphilis, chancroid, lymphogranuloma venereum.
Associated Conditions: Oral ulcers (half of cases), inflammatory bowel diseases (Crohn disease [most common], ulcerative colitis), hematologic diseases (myeloproliferative disorders, cyclic neutropenia, lymphopenia), Behçet syndrome, human immunodeficiency virus (HIV) infection.
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