Rare Rheumatology News

Disease Profile

Ledderhose disease

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

M72.2

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)

Lederhose disease

Categories

Rare Cancers; Skin Diseases

Summary

Ledderhose disease is a type of plantar fibromatosis characterized by the growth of hard and round or flattened nodules (lumps) on the soles of the feet.[1] It is generally seen in middle-aged and elderly people, and occurs in men about 10 times more often than in women. It typically affects both feet and progresses slowly, but not indefinitely.[1] The nodules are often painless at first, but may cause pain when walking as they grow.[2] People with Ledderhose disease may also have other conditions associated with the formation of excess fibrous connective tissue such as Dupuytren contracture, knuckle pads, or Peyronie disease. Repeated trauma, long-term alcohol consumption, chronic liver disease, diabetes, and epilepsy have also been reported in association with this condition. The exact cause of Ledderhose disease is not known, but heredity is thought to play a role in many cases.[1] Treatment, if needed, may involve conservative management, steroid injections, radiotherapy, or surgery (fasciectomy and removal of the fibrous tissue). The condition has a good prognosis, although slow progression is not uncommon. Fasciectomy has been shown to reduce the rate of recurrence.[1][3]

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
Arthralgia
Joint pain
0002829
Lack of skin elasticity
0100679
Paresthesia
Pins and needles feeling
Tingling

[ more ]

0003401
30%-79% of people have these symptoms
Subcutaneous nodule
Firm lump under the skin
Growth of abnormal tissue under the skin

[ more ]

0001482

Treatment

Treatment options may include conservative management, radiation therapy, steroid injections and surgery. Conservative management may include using soft inserts in shoes. It may help to cut holes in the inserts or inner soles to reduce pressure on the nodules.[4] Radiation therapy may help to stop disease progression in early stages, when nodules are very small. It may also help to shrink the size of larger nodules to relieve pain.[4] Corticosteroid injections may be beneficial in the early stages of the disease, when there is no pain or minimal pain.[1][4][5] If or when steroids do not help, surgery may be needed. Surgical options include fasciectomy and excision (removal) of the fibrous tissue. Fasciectomy has been shown to reduce the rate of recurrences.[1] However, recurrence following surgery remains common.[6] 

The International Dupuytren Society provides further information on treatment options for Ledderhose disease.

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.

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

        References

        1. Hougeir FG. Plantar Fibromatosis. Medscape Reference. May 30, 2018; https://emedicine.medscape.com/article/1061903-overview.
        2. Ledderhose disease. International Dupuytren Society. December 28, 2016; https://www.dupuytren-online.info/morbus_ledderhose.html.
        3. Akdag O, Yildiran G, Karamese M & Tosun Z. Dupuytren-Like Contracture of the Foot: Ledderhose Disease.. The Surgery Journaloi:10.1055/s-0036-1593355.. 2016; 2(3):102-104. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5553473/.
        4. Therapies for Ledderhose's disease (Plantar fibromatosis or Morbus Ledderhose). International Dupuytren Society. 2016; https://www.dupuytren-online.info/ledderhose_therapies.html.
        5. Kan HJ, Hovius SE. Long-term follow-up of flaps for extensive Dupuytren's and Ledderhose disease in one family. J Plast Reconstr Aesthet Surg. 2012 Dec;65(12):1741-5; https://www.ncbi.nlm.nih.gov/pubmed/22917697.
        6. Sheon RP, De Keyser F. Ganglia and nodules. In: Basow DS. UpToDate. Waltham, MA: UpToDate; 2013;

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