Porokeratosis: Difference between revisions

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==Overview==
==Overview==
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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>
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 plantaris discreta]]
:* [[Porokeratosis plantaris discreta]]
==Pathophysiology==
[[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''.


==Causes==
==Causes==
The aetiology 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.  
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==
==Risk Factors==
*Sun exposure
* Sun exposure
*Virus infection
* Virus infection
*Immunosuppression
* Immunosuppression
*Radioactivity
* Radioactivity


==Epidemiology and Demographics==
==Epidemiology and Demographics==
It frequently appears in childhood but may appear at any age, especially in nonhereditary cases, with a higher incidence in males
It frequently appears in childhood but may appear at any age, especially in nonhereditary cases, with a higher incidence in males.


==Diagnosis==
==Diagnosis==
===History and Symptoms===
===Physical Findings===
===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]].


Classic porokeratosis Mibelli consists of a single plaque or a small number of plaques of variable size, usually located unilaterally on limbs. . DSP is a variant of PM characterized by small erythematous or pigmented keratotic papules with central atrophy, located on the trunk, genitals, palms, and soles
===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.


==References==
==References==

Revision as of 20:00, 30 July 2013

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Mugilan Poongkunran M.B.B.S [2]

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]

Historical Perspective

Porokeratosis, a disorder of epidermal keratinization was first described by Mibelli in 1893. Since then it has been classified into 6 types.

Classification

Porokeratosis may be divided into the following clinical types:

However, additional types have also been described:[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.

References

  1. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  2. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.


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