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Disease Profile

Lymphomatoid papulosis

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

All ages

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ICD-10

C86.6

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)

LyP

Categories

Blood Diseases; Rare Cancers; Skin Diseases

Summary

Lymphomatoid papulosis (LyP) is a non-contagious, chronic skin condition characterized by the eruption of recurring, self-healing bumps (lesions) on the skin.[1] The lesions typically begin small and then become larger, and they may bleed or ulcerate before becoming scaly and crusty.[1][2] They often develop a red-brown color.[1] Symptoms associated with lesions may include itching and/or pain, which may be debilitating.[1][2] The frequency of eruptions varies over time and from person to person. Lesions may be very persistent, or they may go away for long periods of time before coming back.[1] They may occur anywhere on the body, but they typically develop on the trunk, arms, and legs, and may develop on the hands, face, and genitalia.[2][3] They generally go away on their own over a period of weeks to months (usually between 3 and 8 weeks), with or without scarring.[2] LyP is not contagious.[1][2] The cause of LyP is not known, but there is no evidence that it is hereditary.[1] Stress is often reported to trigger eruptions of lesions.[1] The duration of the condition varies from person to person and may last months, years or decades.[2]

A diagnosis of LyP requires evaluating the symptoms and having a skin biopsy for various types of laboratory tests. While LyP usually is not classified as a cancer (although there has been some debate), it has characteristics of lymphoma under the microscope, and people with LyP have a life-long increased risk of developing lymphoma such as mycosis fungoides, PC-ALCL, or Hodgkin lymphoma.[1][2][3] In 5 to 20 percent of people with LyP, the condition is either preceded by lymphoma, associated with lymphoma, or followed by lymphoma.[2][3] When LyP is diagnosed it is important to rule out these cancers, and for this reason, various blood tests or imaging studies may also be recommended.[2]

Treatment for LyP may speed up the healing of existing lesions or prevent new lesions from forming, but it does not change the overall course or duration of the condition.[2] People with only a few lesions or with no major symptoms or cosmetic concerns may opt to forego treatment. If treatment is desired, topical corticosteroids are an option. Options for people with extensive skin lesions or debilitating symptoms may include topical steroids, phototherapy, oral or topical retinoids, methotrexate, or other medications (alone or in combination).[1][2] Tetracycline is typically used if ulcerated lesions become infected.[4] In most people with LyP-associated lymphomas, treatment of the lymphoma will also clear the LyP.[1]

The long-term outlook (prognosis) in more than 90% of people with LyP is positive, as it usually does not affect overall health.[1] People with LyP who do not develop cancer have a normal life expectancy. While there is a substantial risk to develop lymphoma, the reported mortality rates from associated lymphomas are low.[2]

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 Supporting this Disease

    Social Networking Websites

      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.
      • The Cutaneous Lymphoma Foundation has an information page on lymphomatoid papulosis. Click on The Cutaneous Lymphoma Foundation to view the page.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
        • 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 Lymphomatoid papulosis. Click on the link to view a sample search on this topic.

          References

          1. Lymphomatoid Papulosis. Cutaneous Lymphoma Foundation. https://www.clfoundation.org/lymphomatoid-papulosis. Accessed 8/8/2018.
          2. Kadin ME. Lymphomatoid Papulosis. UpToDate. Waltham, MA: UpToDate; November 29, 2017; https://www.uptodate.com/contents/lymphomatoid-papulosis.
          3. Zic JA. Lymphomatoid Papulosis. Medscape Reference. October 4, 2017; https://emedicine.medscape.com/article/1098954-overview.
          4. Milne O, Newland K, Prince HM, McCormack C, McKay C. Lymphomatoid Papulosis. Australasian College of Dermatologists. June, 2015; https://www.dermcoll.edu.au/atoz/lymphomatoid-papulosis/.