Rare Pediatrics News
Disease Profile
Linear porokeratosis
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
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Age of onset
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ICD-10
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Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Congenital facial linear porokeratosis (type)
Summary
Linear porokeratosis is a rare skin condition characterized by streaks of reddish-brown patches surrounded by a ridge-like border.[1] The patches usually develop in infants or young children, but they sometimes develop in adults.[2] Patches may be on one area of the body (localized) or on multiple areas (generalized). They usually develop in a pattern over the skin known as the "lines of Blaschko," and most commonly occur on the arms or trunk.[2][1] Some people experience itching or pain where the patches occur.[2]
Linear porokeratosis is thought be a form of disseminated superficial actinic porokeratosis (DSAP). Researchers think that people with linear porokeratosis may have one
Treatment options depend on each person's symptoms. There are no specific recommendations for treatment because no randomized
Treatment
- Topical therapies such as 5-fluorouracil (5-FU), imiquimod, retinoids, and calcipotriol.
- Oral retinoids.
- Cryotherapy.
- Electrodessication.
- Surgical removal.
Linear porokeratosis can progress to skin
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
In-Depth Information
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Linear porokeratosis. Click on the link to view a sample search on this topic.
References
- Prak AH. Porokeratosis. Medscape Reference. August 7, 2017; https://emedicine.medscape.com/article/1059123-overview.
- Spencer LV. Porokeratosis. UpToDate. Waltham, MA: UpToDate; September 1, 2016; https://www.uptodate.com/contents/porokeratosis.
- Sommerlad M, Lock A, Moir G, McGregor J, Bull R, Cerio R, Harwood C. Linear porokeratosis with multiple squamous cell carcinomas successfully treated by electrochemotherapy. Br J Dermatol. December, 2016; 175(6):1342-1345. https://www.ncbi.nlm.nih.gov/pubmed/27086492.
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