Difference between revisions of "Lichen sclerosus"

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Lichen sclerosus (view source)

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#redirect [[Vulva#Lichen_sclerosus]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Lichen_sclerosus_-_high_mag.jpg
| Width      =
| Caption    = Lichen sclerosus. [[H&E stain]].
| Synonyms  = balanitis xerotica obliterans (old term for lesion on glans penis)
| Micro      = fibrosis of dermis with loss of adnexal structures - '''key feature''', loss of the rete ridges, (severe) [[hyperkeratosis]], inflammation - often with eosinophils
| Subtypes  =
| LMDDx      = [[morphea profunda]], [[differentiated vulvar intraepithelial neoplasia]], [[lichen planus]], cutaneous [[amyloidosis]], [[malignant melanoma]] with regression
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[vulva]], [[penis]], [[urethra]]
| Assdx      = [[differentiated vulvar intraepithelial neoplasia]], [[vulvar squamous cell carcinoma]]
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  = pruritus
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = chronic, benign
| Other      =
| ClinDDx    =
}}
{{ Infobox external links
| Name          = {{PAGENAME}}
| EHVSC          = 9993
| pathprotocols  =
| wikipedia      =
| pathoutlines  =
}}
'''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'''.<ref name=pmid12602704>{{cite journal |author=Finkbeiner AE |title=Balanitis xerotica obliterans: a form of lichen sclerosus |journal=South. Med. J. |volume=96 |issue=1 |pages=7–8 |year=2003 |month=January |pmid=12602704 |doi= |url=}}</ref><ref name=pmid24268357>{{Cite journal  | last1 = Stewart | first1 = L. | last2 = McCammon | first2 = K. | last3 = Metro | first3 = M. | last4 = Virasoro | first4 = R. | title = SIU/ICUD Consultation on Urethral Strictures: Anterior urethra-lichen sclerosus. | journal = Urology | volume = 83 | issue = 3 Suppl | pages = S27-30 | month = Mar | year = 2014 | doi = 10.1016/j.urology.2013.09.013 | PMID = 24268357 }}</ref>
 
==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.<ref name=pmid9491669>{{Cite journal  | last1 = Kini | first1 = U. | title = Squamous cell carcinoma of the vulva in association with mixed vulvar dystrophy. A brief report with review of literature. | journal = Indian J Cancer | volume = 34 | issue = 2 | pages = 92-5 | month = Jun | year = 1997 | doi =  | PMID = 9491669 }}</ref>
 
==Microscopic==
Features:<ref>URL: [http://www.pathologyoutlines.com/vulva.html#lichensclerosis http://www.pathologyoutlines.com/vulva.html#lichensclerosis]. Accessed on: 19 April 2011.</ref>
*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).<ref>URL: [http://www.webpathology.com/image.asp?n=2&Case=538 http://www.webpathology.com/image.asp?n=2&Case=538]. Accessed on: 25 August 2011.</ref>
*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.<ref name=pmid9537476>{{Cite journal  | last1 = Fung | first1 = MA. | last2 = LeBoit | first2 = PE. | title = Light microscopic criteria for the diagnosis of early vulvar lichen sclerosus: a comparison with lichen planus. | journal = Am J Surg Pathol | volume = 22 | issue = 4 | pages = 473-8 | month = Apr | year = 1998 | doi =  | PMID = 9537476 }}</ref>
 
Notes:
*May have [[angiokeratoma]]-like changes.<ref name=pmid19187108>{{Cite journal  | last1 = Luzar | first1 = B. | last2 = Neil | first2 = SM. | last3 = Calonje | first3 = E. | title = Angiokeratoma-like changes in extragenital and genital lichen sclerosus. | journal = J Cutan Pathol | volume = 36 | issue = 5 | pages = 540-2 | month = May | year = 2009 | doi = 10.1111/j.1600-0560.2008.01091.x | PMID = 19187108 }}</ref>
 
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.<ref name=pmid9537476/>
*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).<ref name=pmid14631186>{{Cite journal  | last1 = Citarella | first1 = L. | last2 = Massone | first2 = C. | last3 = Kerl | first3 = H. | last4 = Cerroni | first4 = L. | title = Lichen sclerosus with histopathologic features simulating early mycosis fungoides. | journal = Am J Dermatopathol | volume = 25 | issue = 6 | pages = 463-5 | month = Dec | year = 2003 | doi =  | PMID = 14631186 }}</ref>
 
====Images====
<gallery>
Image:Lichen_sclerosus_-_low_mag.jpg | Lichen sclerosus - low mag. (WC/Nephron)
Image:Lichen_sclerosus_-_high_mag.jpg | Lichen sclerosus - high mag. (WC/Nephron)
Image:Lichen_sclerosus_-_very_high_mag.jpg | Lichen sclerosus - very high mag. (WC/Nephron)
</gallery>
www:
*[http://www.flickr.com/photos/euthman/2329061374/in/set-72057594114099781 Lichen sclerosus + syringoma (flickr.com)].
*[http://www.webpathology.com/image.asp?n=2&Case=538 Lichen sclerosus (webpathology.com)].
 
==Sign out==
<pre>
Foreskin, Circumcision:
- Lichen sclerosus.
- NEGATIVE for malignancy.
</pre>
 
===Block letters===
<pre>
VULVA, BIOPSY:
- LICHEN SCLEROSUS.
</pre>
 
<pre>
FORESKIN, CIRCUMCISION:
- LICHEN SCLEROSUS.
</pre>
 
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]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Vulva]]
[[Category:Gynecologic pathology]]
Michael
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