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Reactive nonsexually related acute genital ulceration
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Reactive nonsexually related acute genital ulceration

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Contributors: Amanda Tillmann, Susan Burgin MD
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Synopsis

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.

Codes

ICD10CM:
N76.6 – Ulceration of vulva

SNOMEDCT:
68640004 – Ulceration of vulva

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Genital herpes – Presents as painful, small, superficial, and clustered ulcerations on an erythematous base; herpes simplex virus polymerase chain reaction (PCR) or direct fluorescent antibody (DFA) positive. 
Inflammatory and autoimmune conditions:
  • Crohn disease – "Knife-cut" ulcers affecting the inguinal and interlabial folds may be associated with gastrointestinal symptoms. 
  • Behçet syndrome – Ulcers are deep, painful, and heal with scarring; there is also a history of recurrent oral aphthae and uveitis. 
  • Pyoderma gangrenosum – Ulcers have a purulent base with undermined edges and demonstrate a prominent neutrophilic infiltrate on histopathology. 
  • Autoimmune bullous disease (eg, pemphigus and cicatricial pemphigoid) – Exceedingly rare conditions that tend to involve other mucosal surfaces and are confirmed with direct immunofluorescence staining for autoantibodies.
Drug reactions:
  • Fixed drug eruptions – Associated with the use of particular medications such as nonsteroidal anti-inflammatory drugs (NSAIDs), acetaminophen, and sulfonamides, among others.
  • Stevens-Johnson syndrome and toxic epidermal necrolysis – Patients are systemically unwell; cutaneous and mucous membrane involvement by blistering lesions; Nikolsky's sign positive.
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Last Reviewed: 06/23/2017
Last Updated: 07/26/2017
Copyright © 2019 VisualDx®. All rights reserved.
Reactive nonsexually related acute genital ulceration
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Reactive nonsexually related acute genital ulceration : Inguinal lymphadenopathy, Vulvar edema, Vulvar pain, Dysuria
Copyright © 2019 VisualDx®. All rights reserved.