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{{Infobox Disease
| Name          = {{PAGENAME}}
| Image          = Porokeratosis DSAP.JPG
| Caption        = A porokeratosis lesion in a patient with [[disseminated superficial actinic porokeratosis]].
| DiseasesDB    = 31356, 33408, 32454, 34839
| ICD10          =
| ICD9          =
| ICDO          =
| OMIM          = 175860, 175800, 175850
| MedlinePlus    =
| eMedicineSubj  = article
| eMedicineTopic = 1059123
| MeshID        = D017499
}}
__NOTOC__
__NOTOC__
{{SI}}
{{CMG}}; {{AE}} {{M.P}} {{KS}}
==Overview==
 
'''Porokeratosis''' is a specific disorder of keratinization that is characterized histologically by the presence of a cornoid lamella, a thin column of closely stacked, parakeratotic cells extending through the [[stratum corneum]] with a thin or absent granular layer.<ref name=FITZPATRICK2003>Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.</ref>{{rp|532}}
 
==Types==
Porokeratosis may be divided into the following clinical types: <ref name=FITZPATRICK2003 />{{rp|532}}
* ''Plaque-type porokeratosis'' (also known as "Classic porokeratosis" and "Porokeratosis of Mibelli"<ref name=BOLOGNIA2007>{{cite book |author=Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages=1668 |isbn=1-4160-2999-0 |oclc= |doi= |accessdate=}}</ref>) is characterized by [[skin lesion]]s that start as small, brownish [[papule]]s that slowly enlarge to form irregular, annular, hyperkeratotic or verrucous plaques.<ref name=FITZPATRICK2003 />{{rp|533}}<ref name=ANDREWS2005>James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. ISBN 0-7216-2921-0.</ref>{{rp|566}} Sometimes they may show gross overgrowth and even horn-like structures may develop. <ref name=SARMA2009>[http://www.ijdvl.com/article.asp?issn=0378-6323;year=2009;volume=75;issue=5;spage=551;epage=551;aulast=Sarma Familial disseminated plaque type porokeratosis with multiple horns and squamous cell carcinoma involving anal skin]</ref> Skin malignancy, although rare, is reported from all types of porokeratosis. [[Squamous cell carcinoma]]e has been reported to develop in Mibelli's type porokeratosis over partianal areas involving anal mucosa. This was the first report mentioning mucosal malignancy in any form of porokeratosis.<ref name=SARMA2009 />
 
<gallery>
 
Image:Porokeratosis of mibelli01.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
Image:Porokeratosis of mibelli02.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
Image:Porokeratosis of mibelli03.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
 
Image:Porokeratosis of mibelli04.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 


{{CMG}}; {{M.P}}
Image:Porokeratosis of mibelli05.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
 
Image:Porokeratosis of mibelli06.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
Image:Porokeratosis of mibelli07.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
 
 
 
Image:Porokeratosis of mibelli08.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
 
Image:Porokeratosis of mibelli09.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
 
 
Image:Porokeratosis of mibelli10.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>
 
 
 
 
 
 
Image:Porokeratosis of mibelli11.jpg|Porokeratosis of mibelli. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/  With permission from Dermatology Atlas.]''<ref name="Dermatology Atlas">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/}}</ref></SMALL></SMALL>


==Overview==
Porokeratosis is a specific disorder of keratinization that is characterized histologically by the presence of a cornoid lamella, a thin column of closely stacked, parakeratotic cells extending through the [[stratum corneum]] with a thin or absent granular layer.<ref name="Fitz2">Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.</ref>


==Historical Perspective==
</gallery>
Porokeratosis, a disorder of epidermal keratinization was first described by Mibelli in 1893. Since then it has been classified into 6 types.


==Classification==
* ''Disseminated superficial porokeratosis'' is a more generalized processes and involves mainly the extremities in a bilateral, symmetric fashion.<ref name=FITZPATRICK2003 />{{rp|533}}  In about 50% of cases, [[skin lesion]]s only develop in sun-exposed areas, and this is referred to as ''disseminated superficial actinic porokeratosis''<ref name=FITZPATRICK2003 />{{rp|533}}
Porokeratosis may be divided into the following clinical types:
:* [[Classic porokeratosis Mibelli]]
:* [[Disseminated superficial actinic porokeratosis]]
:* [[Disseminated superficial porokeratosis]]
:* [[Porokeratosis palmaris et plantaris disseminata]]
:* [[Linear porokeratosis]]
:* [[Punctate porokeratosis]]


However, additional types have also been described:<ref name="Andrews">James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. ISBN 0-7216-2921-0.</ref>
* ''Porokeratosis palmaris et plantaris disseminata'' is characterized by skin lesions that are superficial, small, relatively uniform, and demarcated by a distinct peripheral ridge of no more than 1mm in height.<ref name=FITZPATRICK2003 />{{rp|534}}<ref name=ANDREWS2005/>{{rp|567}}<ref name=BOLOGNIA2007 />{{rp|1668}}
:* [[Porokeratosis plantaris discreta]]


==Causes==
* ''Linear porokeratosis'' is characterized clinically skin lesions are identical to those of classic porokeratosis, including lichenoid papules, annular lesions, hyperkeratotic plaques with central atrophy, and the characteristic peripheral ridge.<ref name=FITZPATRICK2003 /><ref name=ANDREWS2005/>{{rp|567}}<ref name=BOLOGNIA2007 />{{rp|1668}}
The etiology of porokeratosis is still unclear. An autosomal dominant inheritance has been established for classic porokeratosis Mibelli, disseminated superficial actinic porokeratosis, disseminated superficial porokeratosis, porokeratosis palmaris et plantaris disseminata.  


==Risk Factors==
* ''Punctate porokeratosis'' is a skin condition associated with either classic porokeratosis or linear porokeratosis types of porokeratosis, and is characterized by multiple, minute, and discrete punctate, hyperkeratotic, seed-like skin lesions surrounded by a thin, raised margin on the palms and soles.<ref name=FITZPATRICK2003 />{{rp|535}}<ref name=BOLOGNIA2007 />{{rp|1668}}
* Sun exposure
* Virus infection
* Immunosuppression
* Radioactivity


==Epidemiology and Demographics==
* ''Porokeratosis plantaris discreta'' is a skin condition that occurs in adults, with a 4:1 female preponderance, characterized by a sharply marginated, rubbery, wide-based papules.<ref name=ANDREWS2005/>{{rp|213}}  It is also known as "Steinberg's lesion".<ref name="pmid18685058">{{cite journal |author=Lemont H |title=What's your diagnosis? Porokeratosis plantaris discreta (Steinberg's lesion) |journal=J Am Podiatr Med Assoc |volume=98 |issue=4 |pages=337–8 |year=2008 |pmid=18685058 |doi= |url=http://www.japmaonline.org/cgi/pmidlookup?view=long&pmid=18685058}}</ref> It was characterized in 1970.<ref name="pmid5426632">{{cite journal |author=Taub J, Steinberg MD |title=Porokeratosis plantaris discreta, a previously unrecognized dermatopathological entity |journal=Int. J. Dermatol. |volume=9 |issue=2 |pages=83–90 |year=1970 |pmid=5426632 |doi=10.1111/j.1365-4362.1970.tb04584.x |url=}}</ref>
It frequently appears in childhood but may appear at any age, especially in nonhereditary cases, with a higher incidence in males.


==Diagnosis==
==Pathology==
===Physical Findings===
[[File:Porokeratosis - high mag.jpg|thumb|right|[[Micrograph]] of a case of porokeratosis showing a characteristic cornoid lamella (dark pink/red structure in the right/upper portion of the image). [[H&E stain]].]]
* Classic porokeratosis Mibelli consists of a single plaque or a small number of plaques of variable size, usually located unilaterally on limbs.
* Disseminated superficial porokeratosis is a variant of classic porokeratosis Mibelli characterized by small erythematous or pigmented keratotic papules with central atrophy, located on the trunk, genitals, palms, and soles.
* Linear porokeratosis usually arises as plaques that follows [[Blaschko's lines]].


===Laboratory Findings===
Porokeratosis has a characteristic histomorphologic feature known as a ''cornoid lamella''.
Histopathological examination reveals typical cornoid lamella confirming the diagnosis of porokeratosis.


==Treatment==
==See also==
Skin care with topical isotretinoin 0.1% cream once daily and 5% urea.
* [[List of cutaneous conditions]]
* [[List of cutaneous conditions associated with increased risk of nonmelanoma skin cancer]]


==References==
==References==
{{Reflist|2}}
{{reflist}}


{{WikiDoc Help Menu}}
==External References==
{{WikiDoc Sources}}
[http://www.DSAPinstitute.org www.DSAPinstitute.org]
[[Category:Genodermatoses]]

Latest revision as of 19:37, 30 July 2015

Porokeratosis
File:Porokeratosis DSAP.JPG
A porokeratosis lesion in a patient with disseminated superficial actinic porokeratosis.
OMIM 175800, 175850 175860, 175800, 175850
DiseasesDB 33408, 32454, 34839.htm 31356, 33408, 32454, 34839
eMedicine article/1059123 
MeSH D017499

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mugilan Poongkunran M.B.B.S [2] Kiran Singh, M.D. [3]

Overview

Porokeratosis is a specific disorder of keratinization that is characterized histologically by the presence of a cornoid lamella, a thin column of closely stacked, parakeratotic cells extending through the stratum corneum with a thin or absent granular layer.[1]:532

Types

Porokeratosis may be divided into the following clinical types: [1]:532

  • Plaque-type porokeratosis (also known as "Classic porokeratosis" and "Porokeratosis of Mibelli"[2]) is characterized by skin lesions that start as small, brownish papules that slowly enlarge to form irregular, annular, hyperkeratotic or verrucous plaques.[1]:533[3]:566 Sometimes they may show gross overgrowth and even horn-like structures may develop. [4] Skin malignancy, although rare, is reported from all types of porokeratosis. Squamous cell carcinomae has been reported to develop in Mibelli's type porokeratosis over partianal areas involving anal mucosa. This was the first report mentioning mucosal malignancy in any form of porokeratosis.[4]
  • Disseminated superficial porokeratosis is a more generalized processes and involves mainly the extremities in a bilateral, symmetric fashion.[1]:533 In about 50% of cases, skin lesions only develop in sun-exposed areas, and this is referred to as disseminated superficial actinic porokeratosis[1]:533
  • Porokeratosis palmaris et plantaris disseminata is characterized by skin lesions that are superficial, small, relatively uniform, and demarcated by a distinct peripheral ridge of no more than 1mm in height.[1]:534[3]:567[2]:1668
  • Linear porokeratosis is characterized clinically skin lesions are identical to those of classic porokeratosis, including lichenoid papules, annular lesions, hyperkeratotic plaques with central atrophy, and the characteristic peripheral ridge.[1][3]:567[2]:1668
  • Punctate porokeratosis is a skin condition associated with either classic porokeratosis or linear porokeratosis types of porokeratosis, and is characterized by multiple, minute, and discrete punctate, hyperkeratotic, seed-like skin lesions surrounded by a thin, raised margin on the palms and soles.[1]:535[2]:1668
  • Porokeratosis plantaris discreta is a skin condition that occurs in adults, with a 4:1 female preponderance, characterized by a sharply marginated, rubbery, wide-based papules.[3]:213 It is also known as "Steinberg's lesion".[6] It was characterized in 1970.[7]

Pathology

File:Porokeratosis - high mag.jpg
Micrograph of a case of porokeratosis showing a characteristic cornoid lamella (dark pink/red structure in the right/upper portion of the image). H&E stain.

Porokeratosis has a characteristic histomorphologic feature known as a cornoid lamella.

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  2. ↑ 2.0 2.1 2.2 2.3 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. p. 1668. ISBN 1-4160-2999-0.
  3. ↑ 3.0 3.1 3.2 3.3 James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  4. ↑ 4.0 4.1 Familial disseminated plaque type porokeratosis with multiple horns and squamous cell carcinoma involving anal skin
  5. ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 "Dermatology Atlas".
  6. ↑ Lemont H (2008). "What's your diagnosis? Porokeratosis plantaris discreta (Steinberg's lesion)". J Am Podiatr Med Assoc. 98 (4): 337–8. PMID 18685058.
  7. ↑ Taub J, Steinberg MD (1970). "Porokeratosis plantaris discreta, a previously unrecognized dermatopathological entity". Int. J. Dermatol. 9 (2): 83–90. doi:10.1111/j.1365-4362.1970.tb04584.x. PMID 5426632.

External References

www.DSAPinstitute.org

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