Lichen sclerosus

Lichen sclerosus
Diagnosis in short
Lichen sclerosus. H&E stain.
Synonyms	balanitis xerotica obliterans (old term for lesion on glans penis)
LM	fibrosis of dermis with loss of adnexal structures - key feature, loss of the rete ridges, (severe) hyperkeratosis, inflammation - often with eosinophils
LM DDx	morphea profunda, differentiated vulvar intraepithelial neoplasia, lichen planus, cutaneous amyloidosis, malignant melanoma with regression
Site	vulva, penis, urethra
Associated Dx	differentiated vulvar intraepithelial neoplasia, vulvar squamous cell carcinoma
Symptoms	pruritus
Prognosis	chronic, benign

Lichen sclerosus
External resources
EHVSC	9993

Lichen sclerosus is a relatively common chronic condition classically associated with the vulva. On the vulva is also known as chronic atrophic vulvitis.

On the glans penis it was referred to as balanitis xerotica obliterans, abbreviated BXO.

General

• Associated with differentiated vulvar intraepithelial neoplasia - important.
  • Approximately 50% of vulvar cancer associated with lichen sclerosus.

Clinical:

• Pruritus -> leads to scratching.
• Chronic condition.
• Usu. post-menopausal women.
• May lead to labial fusion.

Treatment:

• Steroids - high dose initially, then a maintenance therapy to prevent relapse.

Notes:

• Mixed vulvar dystrophy = lichen sclerosus + squamous cell hyperplasia.

Microscopic

Features:

• Loss of rete ridges.
• Severe hyperkeratosis.
  • Hyperkeratosis = stratum corneum thickened.
• Fibrosis of the superficial dermis with loss of adnexal structures - key feature.
  • Superficial dermis appears pale and homogeneous (distinct collagen bundles are lost).
• Lichenoid inflammatory infiltrate - often with eosinophils; may destroy the dermal-epidermal junction leading to blister formation.
  • May be prominent - in the inflammatory phase of the disease.

Notes:

• May have angiokeratoma-like changes.

DDx:

• Morphea profunda - deep fibrosis.
• Differentiated vulvar intraepithelial neoplasia - commonly co-exists with lichen sclerosus.
• Lichen planus (LP) - esp. for the inflammatory phase of lichen sclerosus.
  • LP has wedge shaped hypergranulosis, lacks basilar exocytosis, no epidermal atrophy.
• Cutaneous amyloidosis - classically has "cracked" appearance.
• Malignant melanoma with regression - esp. for the inflammatory phase of lichen sclerosus.
• Mycosis fungoides - the inflammatory infiltrate of lichen sclerosis can closely mimic mycosis fungoides (which almost never presents in a genital area).

Images

• 

  Lichen sclerosus - low mag. (WC/Nephron)

• 

  Lichen sclerosus - high mag. (WC/Nephron)

• 

  Lichen sclerosus - very high mag. (WC/Nephron)

www:

• Lichen sclerosus + syringoma (flickr.com).
• Lichen sclerosus (webpathology.com).

Sign out

Foreskin, Circumcision:
	- Lichen sclerosus.
	- NEGATIVE for malignancy.

Block letters

VULVA, BIOPSY:
- LICHEN SCLEROSUS.

FORESKIN, CIRCUMCISION:
- LICHEN SCLEROSUS.

Note:

• BXO afflicts the glans penis.

Micro

Inflammatory phase of lichen sclerosus

The sections show skin with a lymphoplasmacytic predominant interface dermatitis with hyperkeratosis. Spongiosis is present. Scattered inflammatory cell are found with the basal aspect of the epidermis; however, they do not form clusters. No mitotic activity is appreciated.

Focal hypergranulosis and focal parakeratosis is present. Numerous Civatte bodies are identified.

The focal hypergranulosis is not wedge-shaped. There are no pointed rete ridges. There is no basal squamatization.

Sclerotic phase of lichen sclerosus

The sections show skin with loss of the rete ridges, hyperkeratosis and marked fibrosis of the superficial dermis. Few, scattered lymphocytes are seen in the dermis.

A granular layer is present. There is no basal nuclear atypia. There is no acanthosis.

Sclerotic phase of lichen sclerosus with active inflammation

The sections show skin with loss of the rete ridges, a thin epidermis, hyperkeratosis and marked fibrosis of the superficial dermis. Numerous lymphocytes are seen scattered between the collagen fibres in the deeper aspect of the dermis.

A granular layer is present. There is no basal nuclear atypia.

See also

• Vulva.
• Penis.
• Plasma cell vulvitis.
• Plasma cell balanitis.