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Lichen sclerosus

Authors: Jutta Huvila, M.D., Ph.D., C. Blake Gilks, M.D.

Editorial Board Member: Ricardo R. Lastra, M.D.

Deputy Editor-in-Chief: Jennifer A. Bennett, M.D.

Last author update: 13 January 2022

Last staff update: 13 January 2022

Copyright: 2002-2024, PathologyOutlines.com, Inc.

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

Table of Contents

Cite this page: Huvila J, Gilks CB. Lichen sclerosus. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/vulvalichensclerosus.html. Accessed December 21st, 2024.

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

Typically seen in postmenopausal women (Climacteric 2017;20:339)
Associated with other autoimmune disorders (J Eur Acad Dermatol Venereol 2010;24:1031)

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

Etiology

Autoimmune (Int J Biol Sci 2019;15:1429)
May be familial (J Eur Acad Dermatol Venereol 2010;24:1031)

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

44 year old woman with bullous lichen sclerosus (Dermatol Online J 2018;24:13030)
50 year old woman with lichen sclerosus undergoing treatment with nivolumab (Dermatol Online J 2020;26:13030)
60 and 61 year old women with lichen sclerosus with vaginal involvement (Facts Views Vis Obgyn 2017;9:171)
66 year old woman with lichen sclerosus after treatment for vulvar squamous cell carcinoma (Gynecol Oncol Rep 2017;20:73)

Treatment

Topical corticosteroids (first line), with topical calcineurin inhibitor therapy as second line (Am J Clin Dermatol 2013;14:27)

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