Vulva, vagina & female urethra
Nontumor
Lichen sclerosus
Author: Monika Roychowdhury, M.D.
Topic Completed: 1 March 2013
Minor changes: 8 July 2021
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Vulvar lichen sclerosus [title] "loattrfull text"[sb]
Table of Contents
Definition / general | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosis | Additional referencesCite this page: Roychowdhury M. Lichen sclerosus. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/vulvalichensclerosus.html. Accessed September 20th, 2021.
Definition / general
- Chronic lymphocyte mediated skin disease, first described in 1887 (J Urol 2007;178:2268)
- Persistent / progressive inflammatory dermatosis of unknown etiology with predilection for vulva; may be perianal
- Usually age 40 years+ (more common after menopause)
- Rare in children, may involute spontaneously at puberty
- May be familial; associated with autoimmune diseases
- Very itchy and scratchy skin
- Not a premalignant lesion but associated with well differentiated squamous cell carcinoma; in one study, 9% developed VIN, 21% developed invasive squamous cell carcinoma mean 4 years later (Hum Pathol 1998;29:932)
Treatment
- Recognition and treatment of early stages may cause longstanding remission
- Topical steroids (first line) followed by oral or topical retinoids (second line)
- Lifetime surveillance because of increased risk of squamous cell carcinoma (Drugs Aging 2009;26:803)
- Biopsy recommended to rule out squamous cell carcinoma
Clinical images
Images hosted on other servers:
"Cigarette paper" appearance
Clitoris becomes buried under clitoral hood
Gross description
- Atrophic skin resembles cigarette paper (crinkly atrophy); often with loss of labia
- Also white / red ill defined patches
- No vaginal involvement
Microscopic (histologic) description
- Severe hyperkeratosis; thin epidermis, loss of rete pegs, basal cell degeneration, homogenized band of dense fibrosis at papillary dermis, upper dermal edema, chronic inflammation around vessels (particularly eosinophils), often band-like
- 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 with / without dystrophic hair and basement membrane thickening
- Early dermal changes are homogenized collagen and wide ectatic capillaries in dermal papillae immediately beneath basement membrane
- Lymphocytic infiltrate can be sparse or dense, lichenoid or interstitial with epidermal lymphocyte exocytosis and lymphocytic / lymphohistiocytic vasculitis
- Proposed minimal histologic criteria: 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)
Microscopic (histologic) images
Images hosted on other servers:
Various images
Differential diagnosis
Additional references
