Vulva
Nonneoplastic disorders
Lichen sclerosus

Author: Monika Roychowdhury, M.D. (see Authors page)

Revised: 3 October 2017, last major update March 2013

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Vulvar lichen sclerosus [title] "loattrfull text"[sb]

Cite this page: Roychowdhury, M. Lichen sclerosus. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvalichensclerosus.html. Accessed October 20th, 2017.
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