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Plasma cell vulvitis - Anogenital in
Other Resources UpToDate PubMed

Plasma cell vulvitis - Anogenital in

Contributors: Vivian Wong MD, PhD, Mitchell Linder MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Plasma cell vulvitis, or Zoon disease, refers to an extremely rare skin condition where, secondary to inflammation, the vulva becomes irritated, leading to patient complaints of itching and discomfort.

The etiology is unknown but is thought to be related to viral elements, trauma, and/or chronic irritation. The condition tends to be diagnosed in postmenopausal patients.

Patients can present with long-standing complaints of mild itching, pain, burning, and/or bleeding of the vulva. Symptoms may coincide with intercourse. Often, patients report just that a specific area of the vulva is uncomfortable or annoying. Many patients will be asymptomatic and unaware of any issues.

Codes

ICD10CM:
N76.3 – Subacute and chronic vulvitis

SNOMEDCT:
238921004 – Plasma cell vulvitis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Lichen sclerosus – Characteristic "figure-of-eight" distribution with fusion / regression of vulvar tissues.
  • Vaginal atrophy – Usually more globally involved and can occur in vagina as well.
  • Extramammary Paget disease
  • Vulvodynia – No definitive lesion present but pain elicited using a cotton-tipped applicator.
  • Lichen planus – Ulcerations will be present.
  • Squamous cell carcinoma in situ
  • Cutaneous squamous cell carcinoma
  • Allergic reaction / Allergic contact dermatitis
  • Irritant contact dermatitis
  • Vulvar intraepithelial neoplasia
  • Genital herpes simplex virus
  • Fixed drug eruption
  • Psoriasis
  • Pemphigus vulgaris
  • Pemphigus foliaceus
  • Mucous membrane pemphigoid
  • Secondary syphilis
  • Recessive Dystrophic epidermolysis bullosa
  • Junctional epidermolysis bullosa

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:07/09/2020
Last Updated:11/28/2021
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Plasma cell vulvitis - Anogenital in
A medical illustration showing key findings of Plasma cell vulvitis : Labia minora/vestibule, Dyspareunia
Lab image of Plasma cell vulvitis - imageId=312203. Click to open in gallery.
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