Vulva, vagina & female urethra

Nontumor

Lichen sclerosus


Editorial Board Member: Ricardo R. Lastra, M.D.
Deputy Editor-in-Chief: Jennifer A. Bennett, M.D.
Jutta Huvila, M.D., Ph.D.
C. Blake Gilks, M.D.

Last author update: 13 January 2022
Last staff update: 13 January 2022

Copyright: 2002-2022, PathologyOutlines.com, Inc.

PubMed Search: Vulvar lichen sclerosus[TI] free full text[SB]

Jutta Huvila, M.D., Ph.D.
C. Blake Gilks, M.D.
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Cite this page: Huvila J, Gilks CB. Lichen sclerosus. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/vulvalichensclerosus.html. Accessed July 3rd, 2022.
Definition / general
  • Immune mediated chronic fibroinflammatory condition of vulvar skin
  • Most commonly postmenopausal at onset; rarely can occur in children
Essential features
  • Lichenoid interface inflammatory reaction
  • Hyalinization and homogenization of the superficial dermal collagen with displacement of inflammatory cells downward, below the abnormal collagen layer
  • Epidermis is usually thin (atrophic)
Terminology
  • Lichen sclerosus et atrophicus
  • Alternative spelling: lichen sclerosis
Epidemiology
Sites
  • Vulvar skin
  • Can involve perianal skin
  • Typically does not involve vaginal mucosa but focal extension from vulvar skin onto the adjacent mucosa may be seen
Pathophysiology
  • Cell mediated immune response with associated degenerative changes of the basal keratinocytes
  • Secondary fibrosis of the superficial dermis, leading to a subepithelial hypocellular band of homogenous appearing collagen
Clinical features
  • Intensely pruritic
  • May become excoriated
  • Associated with increased risk of developing human papillomavirus (HPV) independent vulvar intraepithelial neoplasia (VIN) and squamous cell carcinoma (Int J Cancer 2017;140:1998)
  • Vulvar skin becomes thinned (cigarette paper appearance), with destruction of normal anatomic landmarks as the disease progresses
Diagnosis
  • Characteristic clinical findings are highly suggestive but a biopsy can provide a definitive diagnosis if there is diagnostic uncertainty and also rule out neoplasia (e.g., VIN or Paget disease)
Prognostic factors
  • Early diagnosis and treatment may prevent disease progression
Case reports
Treatment
Clinical images

Images hosted on other servers:

Cigarette paper appearance

Clitoris becomes buried under clitoral hood

Gross description
  • Diagnosis is made based on small biopsy specimens
  • May be an incidental finding in a resection specimen (e.g., for vulvar squamous cell carcinoma)
Microscopic (histologic) description
  • Vacuolar interface reaction pattern in conjunction with dermal sclerosis (homogenized and hyalinized eosinophilic collagen bundles) of any thickness intervening between inflammatory infiltrate and epithelium or vessel walls (Mod Pathol 1998;11:844)
  • Early lesions show only the inflammation and no or minimal fibrosis (inflammatory phase); the histopathological findings at this stage of disease development are not diagnostic
  • Severe hyperkeratosis; thin epidermis, loss of rete pegs, basal cell degeneration, homogenized band of dense fibrosis at papillary dermis, upper dermal edema, band-like chronic inflammation
  • In early stages, findings are subtle and often more prominent in adnexal structures than in interfollicular skin; adnexal structures show acanthosis, luminal hyperkeratosis and hypergranulosis
  • Early dermal changes are homogenized collagen and wide ectatic capillaries in dermal papillae immediately beneath basement membrane
  • Superficial dermal collagen may be wire-like with lymphocyte entrapment (J Cutan Pathol 2015;42:510)
  • Lymphocytic infiltrate can be sparse or dense, lichenoid or interstitial with epidermal lymphocyte exocytosis
  • Erosions or ulceration can occur (J Low Genit Tract Dis 2021;25:255)
Microscopic (histologic) images

Contributed by Jutta Huvila, M.D., Ph.D.

Typical lichen sclerosis

Sclerotic superficial dermis

Edema

Early lichen sclerosus

Interface change

Virtual slides

Images hosted on other servers:

Vulvar lichen sclerosus

Negative stains
  • Wild type pattern staining for p53
Videos

Introduction to lichen sclerosus

Sample pathology report
  • Right labium majus, biopsy:
    • Lichen sclerosus (see comment)
    • Comment: This vulvar biopsy shows established lichen sclerosus. Negative for dysplasia or malignancy.
Differential diagnosis
  • HPV independent vulvar intraepithelial neoplasia (VIN):
    • Other terminology: differentiated VIN (dVIN), differentiated exophytic vulvar intraepithelial lesion (DEVIL), vulvar acanthosis with altered differentiation (VAAD), vulvar altered maturation (VAM)
    • Shows epithelial hyperplasia and loss of normal maturation; often shows significant basal atypia and may show mutant pattern p53 immunostaining (Mod Pathol 2011;24:297)

  • Lichen planus:
    • Clinically, the presence of erosions, oral involvement, a burning sensation or a hyperkeratotic lesional margin favor a diagnosis of lichen planus over lichen sclerosus (Australas J Dermatol 2020;61:324)
    • Typically involves mucosa or nonhair bearing skin
    • Subepithelial band-like inflammatory infiltrate is directly under the squamous epithelium, without a separating area of fibrosis / sclerosis
    • Pointed rete ridges are more common in lichen planus, while the presence of epidermal atrophy or basal lamina thickening favor lichen sclerosus (Am J Surg Pathol 1998;22:473)
    • Early lesions of lichen sclerosus, before the fibrosis becomes established, can be difficult or impossible to distinguish from lichen planus; such cases can be signed out descriptively, indicating that follow up, with or without rebiopsy, should allow for definitive diagnosis
    • Lichen planus can coexist with lichen sclerosus (J Low Genit Tract Dis 2017;21:204)

  • Lichen simplex chronicus:
    • Common; spares the vaginal mucosa (Dermatol Clin 2010;28:669)
    • Epidermal hyperplasia is present, rather than atrophic changes, with no degenerative changes of the basal epithelial layer and no superficial subepidermal sclerosis
    • Excoriation is common and may lead to subepidermal scarring but with variably sized collagen bundles and not the homogenized sclerotic band of lichen sclerosis
    • Spongiosis / spongiotic dermatitis may be present but is not necessary for diagnosis (Int J Womens Dermatol 2017;3:58)
    • Hypergranulosis is common
Board review style question #1

This vulvar biopsy shows

  1. Lichen planus
  2. Lichen simplex chronicus
  3. Lichen sclerosus
  4. Vulvar intraepithelial neoplasia (VIN)
Board review style answer #1
C. Lichen sclerosus

Comment Here

Reference: Lichen sclerosus
Board review style question #2
There is an association between lichen sclerosus of the vulva and

  1. High grade squamous intraepithelial lesion (HSIL / VIN3)
  2. Human papillomavirus (HPV) independent vulvar intraepithelial neoplasia (VIN)
  3. Lichen planus
  4. Psoriasis
Board review style answer #2
B. Human papillomavirus (HPV) independent vulvar intraepithelial neoplasia (VIN)

Comment Here

Reference: Lichen sclerosus
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