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# | {{ 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]] |
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