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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Linear porokeratosis


Other Names for this Disease

  • Congenital facial linear porokeratosis (type)
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Overview

What is linear porokeratosis?

How might linear porokeratosis be treated?

What is linear porokeratosis?

Linear porokeratosis is a skin condition that most often begins in infancy or early childhood, but it can occur at any age.  The main feature of this condition is the development of reddish brown, slightly raised markings on the skin arranged in lines or streaks on one side of the body.  These markings are not usually painful, though they can sometimes cause open sores in the skin.  There is up to an 11% chance that these markings could progress to skin cancer (basal cell cancer or squamous cell carcinoma) over time.  The exact cause of linear porokeratosis is unknown, but risk factors may include exposure to the sun or radiation, problems with the immune system (immunosuppression), or genetic predisposition.[1]
Last updated: 11/17/2011

How might linear porokeratosis be treated?

Because linear porokeratosis is a rare condition, there is no established treatment protocol.  Protection from sun exposure and regular visits to a doctor to check for skin cancer are encouraged as routine care.  Treatment options depend on the size, location, and severity of the characteristic skin markings.  Several medications (5-fluorouracil, acitretin) have been shown to be effective for treating this condition in a small number of patients.[1]  We identified a single report of photodynamic therapy being used to successfully treat an individual with linear porokeratosis.[2]  Surgery is recommended to remove any skin cancer that may develop.[1]
Last updated: 11/17/2011

References
  1. Spencer LV . Porokeratosis. Medscape Reference. May 30, 2012; http://emedicine.medscape.com/article/1059123-overview#a0101. Accessed 8/21/2012.
  2. García-Navarro X, Garcés JR, Baselga E, Alomar A. Linear porokeratosis: excellent response to photodynamic therapy. Archives of Dermatology. 2009; 145:526-527. http://www.ncbi.nlm.nih.gov/pubmed/19451495. Accessed 11/15/2011.


Other Names for this Disease
  • Congenital facial linear porokeratosis (type)
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.