Porokeratosis 

  • Author: Linda V Spencer, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 7, 2010
 

Background

Porokeratosis is a clonal disorder of keratinization characterized by one or more atrophic patches surrounded by a clinically and histologically distinctive hyperkeratotic ridgelike border called the cornoid lamella. Five clinical variants of porokeratosis are recognized: classic porokeratosis of Mibelli (PM), disseminated superficial actinic porokeratosis (DSAP), porokeratosis palmaris et plantaris disseminata (PPPD), linear porokeratosis, and punctate porokeratosis. Several other variants have been described, including hyperkeratosis types, a pruritic papular variant, and a verrucous variant that is localized to the buttocks and mimics psoriasis.[1]

A related disorder is porokeratotic adnexal ostial nevus (PAON). This is a rare disorder of keratinization with eccrine and hair follicle involvement. This name was proposed to incorporate porokeratotic eccrine ostial and dermal duct nevus (PEODDN) and porokeratotic eccrine and hair follicle nevus (PEHFN).[2]

Occasionally, a patient may develop more than one type of porokeratosis simultaneously or consecutively.

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Pathophysiology

Clonal hyperproliferation of atypical keratinocytes leads to the formation of the cornoid lamella, which expands peripherally and forms the raised boundary between abnormal and normal keratinocytes. Local or systemic changes in immune function may allow the development of atypical clones of keratinocytes. Loss of heterozygosity has been proposed as a mechanism for linear porokeratosis associated with a personal or family history of DSAP.

Several risk factors for the development of porokeratosis have been identified; these factors include genetic inheritance, ultraviolet radiation, and immunosuppression. Immunosuppression associated with porokeratosis may be secondary to a disease process such as HIV infection or lymphoma or an iatrogenic suppression such as with immune-modulating drugs used to prevent organ transplant rejection or to treat autoimmune diseases.[3, 4] An autosomal dominant mode of inheritance has been established for familial cases of all forms of porokeratosis. Extensive sun exposure is thought to cause DSAP, although the typical sparing of facial skin is unexplained. Excessive natural or artificial ultraviolet radiation, electron beam therapy, and extensive radiation therapy are well-established trigger factors. Immunosuppression may induce new lesions or cause preexisting lesions to flare.

The formation of squamous or basal cell carcinomas has been reported in all forms of porokeratosis.[5, 6, 7] Chromosomal instability and reduced immune surveillance with overexpression of p53 are hypothesized to play a role in the development of cutaneous malignancies within porokeratosis.[8, 9, 10, 11, 12]

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Epidemiology

Frequency

United States

DSAP is relatively common. The other forms of porokeratosis are rare.

Mortality/Morbidity

  • Most lesions are asymptomatic. Ulcerative lesions have been described. Giant PM in a facial or acral location may cause destruction of underlying soft tissue or pseudoainhum with amputation.[13]
  • Malignant degeneration has been reported in all forms of porokeratosis, with risks of 7.5% and 11% determined from 2 published reviews of the literature. Large lesions, lesions of long-standing duration, and the linear type of porokeratosis were found to be at greatest risk. Extensive metastases of a squamous cell carcinoma developing within a large PM lesion has been reported.[14]

Race

  • Porokeratosis most commonly occurs in fair-skinned individuals. Porokeratosis is rare in darker-skinned populations.

Sex

  • PM and PPPD affect men twice as often as women.
  • DSAP is 3 times as likely to develop in women compared with men.
  • Linear porokeratosis is seen with equal incidence in men and women.

Age

  • PPPD and linear porokeratosis may be seen at any age, from birth to adulthood.
  • PM usually develops in childhood.
  • Disseminated superficial porokeratosis (DSP) generally develops in the third or fourth decade of life.
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Contributor Information and Disclosures
Author

Linda V Spencer, MD  Consulting Staff, Department of Dermatology, St Clare Medical Center

Linda V Spencer, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Indiana State Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Marjan Garmyn, MD, PhD  Professor, Faculty of Medicine, Katholieke Universiteit Leuven, Belgium; Chair and Adjunct Head, Department of Dermatology, University of Leuven, Belgium

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety monitoring committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring committee

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Porokeratosis of Mibelli on the lower leg in a renal transplant recipient.
Disseminated superficial actinic porokeratosis on the lower legs of a female patient.
A 42-year-old woman with multiple lesions on the pretibial aspects of the legs.
A young boy with a linear lesion of porokeratotic eccrine ostial and dermal duct nevus extending onto the nailbed, causing pterygium formation.
 
 
 
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