Rare Pediatrics News

Disease Profile

Lichen planus pigmentosus

Prevalence
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.

Unknown

Age of onset

Adult

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ICD-10

L43.8

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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Other names (AKA)

LP pigmentosus; Lichen planus pigmentosa; LP pigmentosa;

Categories

Skin Diseases

Summary

Lichen planus pigmentosus (LPP) is a rare form of lichen planus. It is characterized by oval or irregularly-shaped brown to gray-brown macules and patches on the skin. Areas that are exposed to sun such as the forehead, temples and neck are most commonly affected. However, the macules and patches may also develop on the trunk or in places where two areas of skin touch or rub together (i.e. the armpit, groin, etc). LPP is a chronic, relapsing condition with periods of worsening symptoms separated by periods of remission (decreasing or disappearing symptoms). The cause of LPP is unknown, but studies suggest it may be triggered by UV light, viral infections, or agents applied to the skin such as mustard oil and amla oil. Treatment for LPP depends on the symptoms in each person.[1][2][3]

Symptoms

Lichen planus pigmentosus (LPP) is characterized by oval or irregularly-shaped brown to gray-brown macules and patches on the skin. Areas that are exposed to sun such as the forehead, temples and neck are most commonly affected. However, the macules and patches may also develop on the trunk or in places where two areas of skin touch or rub together (i.e. the armpit, groin, etc). LPP is a chronic, relapsing condition with periods of exacerbations (worsening symptoms) separated by periods of remission (a decrease in or disappearance of symptoms).[1][2][4]

Although there usually are not other symptoms, some people may experience mild itching and/or burning, or develop other features of lichen planus. Please click here to learn more about the signs and symptoms that may be found in lichen planus.[1]

LPP usually affects young to middle-aged adults who have dark skin, especially those of Indian, Latin American, or Middle Eastern descent.[1]

Cause

The underlying cause of lichen planus pigmentosus is currently unknown. However, studies suggest that the condition may be triggered by viral infections, UV light or the application of certain oils to the hair or skin (i.e. mustard oil, amla oil, or henna hair dyes).[1][2]

Diagnosis

A diagnosis of lichen planus pigmentosus is usually suspected based on the presence of characteristic signs and symptoms. A skin biopsy may then be ordered to confirm the diagnosis.[1][3]

Treatment

There is currently no consensus about treatment for lichen planus pigmentosus (LPP). Management includes avoiding things that make it worse in each case, such as sun exposure or friction in body folds (via weight loss or wearing loose clothing).[5]

No treatment options have shown consistent responses, and evidence of successful treatment has been limited to a few case series.[5] Treatment options may include:[1][4][6][7][8]

In some cases, LPP goes away on its own within weeks. In other cases, pigmentation persists for years.[1][5]

    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.
    • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
    • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
    • PubMed is a searchable database of medical literature and lists journal articles that discuss Lichen planus pigmentosus. Click on the link to view a sample search on this topic.

    References

    1. Vashi N & Kundu R. Approach to the patient with hyperpigmentation disorders. UpToDate. January 2015; https://www.uptodate.com/contents/approach-to-the-patient-with-hyperpigmentation-disorders.
    2. Lichen planus pigmentosus. Orphanet. May 2011; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=254463.
    3. Lichen planus. DermNet NZ. March 2015; https://dermnetnz.org/scaly/lichen-planus.html.
    4. Van R. Lichen planus pigmentosus. Dermatology Online Journal. 19(12):https://escholarship.org/uc/item/1tk5s24q.
    5. Irene Mathews, Devinder Mohan Thappa, Nidhi Singh, Debasis Gochhait. Lichen planus pigmentosus: A short review. Pigment International. 2016; 3(1):5-10. https://www.pigmentinternational.com/article.asp?issn=2349-5847;year=2016;volume=3;issue=1;spage=5;epage=10;aulast=Mathews.
    6. Han XD, Goh CL. A case of lichen planus pigmentosus that was recalcitrant to topical treatment responding to pigment laser treatment. Dermatol Ther. September-October 2014; 27(5):264-267. https://www.ncbi.nlm.nih.gov/pubmed/24796489.
    7. Al-Mutairi N, El-Khalawany M.. Clinicopathological characteristics of lichen planus pigmentosus and its response to tacrolimus ointment: an open label, non-randomized, prospective study. J Eur Acad Dermatol Venereol. May 2010; 24(5):535-540. https://www.ncbi.nlm.nih.gov/pubmed/?term=19840200.
    8. Ghosh A, Coondoo A. Lichen Planus Pigmentosus: The Controversial Consensus. Indian Journal of Dermatology. September-October 2016; 61(5):482-486. https://www.ncbi.nlm.nih.gov/pubmed/27688435.

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