Reactive nonsexually related acute genital ulceration (RNSRAGU) is a rare condition that is most prevalent in sexually inactive, adolescent females, predominantly of Northern European descent. It may be associated with significant pain and emotional distress and is often under-recognized and misdiagnosed.

Although the pathophysiology of RNSRAGU remains yet to be fully characterized, it is suspected that a hypersensitivity reaction to a bacterial or viral infection is largely responsible. It is postulated that the deposition of immune complexes in dermal vessels and the associated inflammation results in ischemia, tissue necrosis, and, ultimately, dermal ulceration. A history of oral aphthosis may represent a potential risk factor for RNSRAGU. It remains uncertain what role the factors that contribute to oral aphthosis, such as vitamin deficiencies, stress, trauma, and hormonal changes, may play in the development of vulvar ulceration.

Most cases are associated with a prodromal illness featuring flu-like or mononucleosis-like symptoms including fever, pharyngitis, lymphadenopathy, transaminitis, myalgia, and fatigue. Viral gastroenteritis has also been reported as a prodromal illness in the literature. Vulvar ulceration tends to be of acute onset, occurring soon after initial signs of pain and discomfort.

The lesions are exquisitely tender; consequently, dysuria is a characteristic complication of RNSRAGU. Associated symptoms of concurrent oral aphthosis, labial edema, and inguinal lymphadenopathy may also be present. In the absence of treatment, the lesions resolve within a matter of weeks and heal without scarring. Approximately one-third of patients will experience recurrent episodes and develop complex aphthosis. These recurrences are not usually associated with a prodrome.