Porokeratosis: Difference between revisions
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==Overview== | ==Overview== | ||
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Porokeratosis may be divided into the following clinical types: | Porokeratosis may be divided into the following clinical types: | ||
:* Classic porokeratosis Mibelli | :* Classic porokeratosis Mibelli | ||
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| OMIM = 175860, 175800, 175850 | |||
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'''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 /> | |||
* ''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 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}} | |||
* ''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}} | |||
* ''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}} | |||
* ''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> | |||
==Pathology== | |||
[[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]].]] | |||
Porokeratosis has a characteristic histomorphologic feature known as a ''cornoid lamella''. | |||
==See also== | |||
* [[List of cutaneous conditions]] | |||
* [[List of cutaneous conditions associated with increased risk of nonmelanoma skin cancer]] | |||
==References== | |||
{{reflist}} | |||
==External References== | |||
[http://www.DSAPinstitute.org www.DSAPinstitute.org] | |||
[[Category:Genodermatoses]] | |||
</gallery> | |||
:* Disseminated superficial actinic porokeratosis | :* Disseminated superficial actinic porokeratosis | ||
:* Disseminated superficial porokeratosis | :* Disseminated superficial porokeratosis | ||
Revision as of 18:38, 25 September 2014
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Mugilan Poongkunran M.B.B.S [2] Kiran Singh, M.D. [3]
Overview
Porokeratosis is a specific disorder of epidermal 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]
Historical Perspective
Porokeratosis was first described by Mibelli in 1893.
Classification
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
- Porokeratosis plantaris discreta[2]
Causes
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
- Sun exposure
- Virus infection
- Immunosuppression
- Radioactivity
Epidemiology and Demographics
It frequently appears in childhood but may appear at any age, especially in nonhereditary cases, with a higher incidence in males.
Diagnosis
Physical Findings
- 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
Histopathological examination reveals typical cornoid lamella confirming the diagnosis of porokeratosis.
Treatment
Skin care with topical isotretinoin 0.1% cream once daily and 5% urea.