Lichen sclerosus

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Lichen sclerosus
Diagnosis in short

Lichen sclerosus. H&E stain.

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

Associated Dx differentiated vulvar intraepithelial neoplasia, vulvar squamous cell carcinoma
Symptoms pruritis
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 glands penis it is referred to as balanitis xerotica obliterans, abbreviated BXO.[1]

General

Clinical:

  • Pruritis -> 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.[2]

Microscopic

Features:[3]

  • 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).[4]
  • 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.[5]

Notes:

DDx:

Images

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VULVA, BIOPSY:
- LICHEN SCLEROSUS.
FORESKIN, CIRCUMCISION:
- LICHEN SCLEROSUS.

Note:

  • BXO afflicts the glands.

Micro

Inflammtory 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

References

  1. Finkbeiner AE (January 2003). "Balanitis xerotica obliterans: a form of lichen sclerosus". South. Med. J. 96 (1): 7–8. PMID 12602704.
  2. Kini, U. (Jun 1997). "Squamous cell carcinoma of the vulva in association with mixed vulvar dystrophy. A brief report with review of literature.". Indian J Cancer 34 (2): 92-5. PMID 9491669.
  3. URL: http://www.pathologyoutlines.com/vulva.html#lichensclerosis. Accessed on: 19 April 2011.
  4. URL: http://www.webpathology.com/image.asp?n=2&Case=538. Accessed on: 25 August 2011.
  5. 5.0 5.1 Fung, MA.; LeBoit, PE. (Apr 1998). "Light microscopic criteria for the diagnosis of early vulvar lichen sclerosus: a comparison with lichen planus.". Am J Surg Pathol 22 (4): 473-8. PMID 9537476.
  6. Luzar, B.; Neil, SM.; Calonje, E. (May 2009). "Angiokeratoma-like changes in extragenital and genital lichen sclerosus.". J Cutan Pathol 36 (5): 540-2. doi:10.1111/j.1600-0560.2008.01091.x. PMID 19187108.
  7. Citarella, L.; Massone, C.; Kerl, H.; Cerroni, L. (Dec 2003). "Lichen sclerosus with histopathologic features simulating early mycosis fungoides.". Am J Dermatopathol 25 (6): 463-5. PMID 14631186.