Rare Oncology News

Disease Profile

Lichen planus pemphigoides

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.

<1 / 1 000 000

US Estimated

Europe Estimated

Age of onset

All ages

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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 pemphigoides

Categories

Skin Diseases

Summary

Lichen planus pemphigoides (LPP) is a rare cross-over syndrome between lichen planus and bullous pemphigoid. Like other forms of lichen planus, it is characterized by a skin rash (shiny, flat-topped, firm bumps that are a purple color and vary from pin point size to larger than a centimeter); however, people affected by LPP subsequently develop blisters on both the lichen planus skin lesions and on normal skin. The exact underlying cause of LPP is currently unknown, but some studies suggest that it may be associated with an allergic or immune reaction. Treatment for LPP is based on the signs and symptoms present in each person.[1][2]

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Hyperkeratosis
0000962
Hypopigmented streaks
0007535
Lichenification
0100725
Skin vesicle
0200037
30%-79% of people have these symptoms
Abnormal oral mucosa morphology
Abnormality of lining of mouth
0011830
Pruritus
Itching
Itchy skin
Skin itching

[ more ]

 0000989
1%-4% of people have these symptoms
Abnormal blistering of the skin
Blistering, generalized
Blisters

[ more ]

 0008066
Abnormality of the nail
0001597
Blepharitis
Inflammation of eyelids
0000498
Conjunctivitis
Pink eye
0000509
Do you have updated information on this disease? We want to hear from you.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

    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.

    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 pemphigoides. Click on the link to view a sample search on this topic.

      References

      1. Lichen planus pemphigoides. Orphanet. May 2011; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=254478.
      2. Tsu-Yi Chuang, MD, MPH. Lichen Planus. Medscape Reference. May 2015; https://emedicine.medscape.com/article/1123213-overview.