Plasma cell vulvitis - Anogenital in
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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.
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
N76.3 – Subacute and chronic vulvitis
SNOMEDCT:
238921004 – Plasma cell vulvitis
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Differential Diagnosis & Pitfalls
- Lichen sclerosus – Characteristic "figure-of-eight" distribution with fusion / regression of vulvar tissues.
- Vulvar 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.
- Bowen disease
- Squamous cell carcinoma
- Allergic reaction / contact dermatitis
- Irritant contact dermatitis
- Vulvar intraepithelial neoplasia
- Herpes simplex
- Fixed drug eruption
- Psoriasis
- Pemphigus vulgaris
- Pemphigus foliaceus
- Mucous membrane pemphigoid
- Secondary syphilis
- Recessive dystrophic epidermolysis bullosa
- Junctional epidermolysis bullosa
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Last Reviewed:07/09/2020
Last Updated:11/28/2021
Last Updated:11/28/2021