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Pruritus vulvae - Management
Which malignant and pre-malignant conditions may cause pruritus vulvae?

  • Malignant neoplasms of the vulva (uncommon).
    • Squamous cell carcinomas account for 90% of malignant disease of the vulva. Other less common neoplastic conditions include peri-anal intraepithelial neoplasia, basal cell carcinoma, melanoma, and carcinoma of Bartholin's gland.
    • Squamous cell carcinomas often arise from pre-existing background disease. Unlike vulval intraepithelial neoplasia (VIN) and Paget's disease, squamous cell carcinomas are rarely itchy; they usually present as a lump or ulcer and are usually tender:
      • In elderly women, the background disease is most likely to be lichen sclerosus or lichen planus.
      • Vulval intraepithelial neoplasia — is a pre-malignant skin lesion of the vulva and is the most likely background disease in young women. This is usually linked to the wart virus.
  • Vulval intraepithelial neoplasia (VIN) is a pre-malignant skin lesion of the vulva.
    • If left untreated, VIN may go away by itself (especially the type of VIN known as 'Bowenoid papulosis'), or it may turn into an invasive cancer in later years. On average, it takes well over 10 years for VIN to progress to cancer.
    • Vulval intraepithelial neoplasia may occur in women of all ages; the average age of women with VIN is 45–50 years of age, although currently an increasing number of younger women (even teenagers) are presenting with the condition.
    • Vulval intraepithelial neoplasia may be completely symptom-free, however most women present with:
      • Mild to severe vulval itching.
      • Mild to severe vulvar burning.
      • One or more slightly raised, well-defined skin lesions that may be pink, red, brown, or white.
    • There are two types of VIN:
      • Usual-type VIN — caused by persistent infection with high-risk human papillomavirus. Risk factors for developing usual type VIN include smoking and immunosuppression.
      • Differentiated-type VIN — associated with lichen sclerosus. This is less common than usual-type VIN (accounts for less than 2–5% of all VIN lesions).
  • Extra mammary Paget's disease (very rare) — a cutaneous neoplasm with a chronic eczema-like rash of the skin around the anogenital regions of males and females. A common symptom is a mild to intense itching of a lesion found around the groin, genitalia, perineum, or peri-anal area. Pain and bleeding may occur from scratching lesions that have been around for a long time. Thickened plaques may form that can become red, scaly, and crusty. Plaques are fixed (unchanging over a few weeks), with sharply demarcated margins and are usually asymmetric, often only affecting one side of the vulva (or peri-anal skin) Although they may appear similar to eczema, they fail to clear up with topical steroid creams.

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