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Disease Profile

Erythema elevatum diutinum

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

L95.1

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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Categories

Blood Diseases; Skin Diseases

Summary

Erythema elevatum diutinum (EED) is a disorder of the skin associated with small blood vessel inflammation (vasculitis). Symptoms include red, purple, brown or yellow bumps of different sizes that grow on or just below the skin. These growths are located mainly on the elbows, knees, ankles, hands, and fingers. People with EED may also have joint pain, but few other symptoms. EED symptoms begin in adulthood and can last for many years. Many cases get better on their own, but this may take many years. The cause of EED is unknown. Diagnosis is made based on the symptoms and a skin biopsy to look at the skin growths. Treatment includes antibiotics and other medications.[1][2]

Symptoms

The following list includes the most common signs and symptoms in people with erythema elevatum diutinum (EED). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

Symptoms may include:[1][3]

  • Yellow, purple, red and brown bumps on the skin (papulesnodules)
  • Red, itchy patches of skin (plaques)
  • Pain or burning 
  • Joint pain (arthralgia)

Symptoms usually begin between ages 40 and 60. Skin changes most often start on the knees, elbows, hands or fingers, and then move to the trunk. The skin changes may increase in size and number over many years. EED can last for several decades and then symptoms start to improve. Recovery is slow and may take 5 to 10 years.[2]

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
Skin rash
0000988
Vasculitis in the skin
0200029
30%-79% of people have these symptoms
Abnormal blistering of the skin
Blistering, generalized
Blisters

[ more ]

0008066
Increased circulating antibody level
0010702
Skin vesicle
0200037
5%-29% of people have these symptoms
Myalgia
Muscle ache
Muscle pain

[ more ]

0003326
Skin nodule
0200036

Cause

The cause of erythema elevatum diutinum is unknown. It has been associated with autoimmune diseases, frequent infections, and blood diseases.[1][2][3]

Treatment

Treatment of erythema elevatum diutinum is based on managing the symptoms. Medications such as anti-inflammatory drugs, antibiotics, and other drugs that fight infections. The skin growths may recur if medication is stopped. In some cases, surgery may be necessary to remove the skin growths.[1][2][4]

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

    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 Erythema elevatum diutinum. Click on the link to view a sample search on this topic.

        References

        1. Doktor V, Hadi A, Hadi A, Phelps R, Goodheart H. Erythema elevatum diutinum: a case report and review of literature. Int J Dermatol. 2019; 58(4):408-415. https://pubmed.ncbi.nlm.nih.gov/30074624.
        2. Newburger J, Schmieder GJ. Erythema Elevatum Diutinum. StatPearls. Treasure Island (FL): StatPearls Publishing. Updated Dec 20, 2019; https://pubmed.ncbi.nlm.nih.gov/28846276.
        3. Mançano VS, Dinato SLME, Almeida JRP, Romiti N. Erythema elevatum diutinum. An Bras Dermatol. 2018; 93(4):614-615. https://pubmed.ncbi.nlm.nih.gov/30066783.
        4. Bansal R, Aron J, Rajnish I. Erythema Elevatum Diutinum. Am J Med Sci. 2017; 353(2):189. https://pubmed.ncbi.nlm.nih.gov/28183422.

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