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Prurigo nodularis


Información en español Title




Other Names:
Nodular prurigo

Summary Summary


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Prurigo nodularis (PN) is a skin disease that causes hard, itchy lumps (nodules) to form on the skin.[1] The itching (pruritus) can be intense, causing people to scratch themselves to the point of bleeding or pain. Scratching can cause more skin lesions to appear.[2][3] The itching is worsened by heat, sweating, or irritation from clothing.[4] In some cases, people with PN have a history of other diseases including eczema (atopic dermatitis), lymphoma, HIV infection, severe anemia, or kidney disease.[1] 

The exact cause of PN is unknown. Although scratching is known to cause more nodules to appear, it is unclear what causes the itching to develop in the first place.[2][3] Diagnosis of the disease is based on observing signs such as extremely itchy skin with the formation of nodules. In some cases, a skin biopsy is used to confirm the diagnosis.[3][4] Treatment may include corticosteroid creams, oral medications, cryotherapy, or photochemotherapy.[1][4][5]
Last updated: 4/19/2018

Symptoms Symptoms


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The main symptom of prurigo nodularis (PN) is the formation of hard, very itchy lumps (nodules) on the skin. The nodules can range in size from very small to about half an inch in diameter. The nodules often have a rough, dry top and can range in number from a few to hundreds. Nodules most commonly form on the outer arms, shoulders, and legs. Nodules can also form on the neck and trunk, and they rarely form on the face and palms. They may be lighter or darker in color than the surrounding skin. Scarring may occur after nodules begin to heal.[1][2][3]  

The symptoms of PN can begin at any age but are most common in adults between 20-60 years.[2] People who have PN may become very concerned about the appearance of the nodules, and the intensely itchy skin may interfere with sleep or with everyday activities. This can cause people with PN to develop stress and depression.[3] 
Last updated: 4/19/2018
Do you have updated information on this disease? We want to hear from you.

Cause Cause


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The exact cause of prurigo nodularis (PN) is not well-understood. It is thought that nodules are more likely to form when skin has been scratched or irritated in some way. Therefore, the act of a person scratching skin can cause the nodules to form. However, the cause of the skin to originally become intensely itchy is unclear. Many people with PN have a history of eczema (atopic dermatitis), other skin conditions, or allergies.[2] 

When people with PN have a skin biopsy, it can be seen that the nerves in the skin are thickened. It is thought that these thickened nerves may send stronger signals to the brain that the skin is itchy. This can cause a person to scratch the skin, which causes more nodules to form and the nerves to become even more thickened. This cycle, called the itch-scratch cycle, is thought to cause an increase in the number of nodules associated with PN. However, exactly why the skin becomes itchy in the first place is unclear and may vary from person to person.[4] 
Last updated: 4/19/2018

Inheritance Inheritance


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In general, prurigo nodularis (PN) is not thought to be passed directly from parents to children. This is because changes in any one specific gene are not thought to cause PN. However, the development of PN is sometimes associated with having other skin diseases, allergies, or other health problems.[3] Many people with PN also have family members who have these same health issues.[2]

If family members of people who have PN have these other health concerns, it may be that they are at an increased risk to develop PN themselves. This is possibly related to shared environmental and genetic factors that cause the underlying skin disease.[2]
Last updated: 4/19/2018

Diagnosis Diagnosis


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Prurigo nodularis may be suspected when a person has signs and symptoms of the disease including intensely itchy skin and the development of nodules on the skin.[4] In some cases, a doctor may wish to perform a skin biopsy to look for signs of the disease such as thickened nerves in the skin.[2] After the diagnosis has been confirmed, other tests such as a blood test and tests of liver and kidney function may be recommended to rule out underlying causes of the disease.[3] 
Last updated: 4/19/2018

Treatment Treatment


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Prurigo nodularis (PN) can be challenging to treat because people with the disease may respond to treatments differently. Due to the intensity of the itch, people with PN may try several different treatments without receiving much relief.  Some people may try all current treatments available without receiving complete relief. For most people, a combination of several treatments may need to be tried to find out what will work best.

The most commonly used treatments for PN are:[1][3][5][6]
  • Corticosteroid creams that are applied to the nodules (topical) and covered with special bandages that are air- and water-tight
  • Corticosteroid injections into the nodules
  • Ointments with menthol or phenol to cool and soothe itchy skin
  • Capsaicin cream
  • Oral corticosteroids
  • Oral antihistamines
  • Selective serotonin reuptake inhibitors (SSRIs)
If these treatments are not effective, other procedures may be used. These include cryotherapy, which uses very cold temperatures to try to reduce the size of the nodules, or laser therapy. Some people have used photochemotherapy, which combines the use of a medication that increases the skin’s sensitivity to ultraviolet (UV) rays with special light therapy. In some cases, immunosuppressants have been used to treat PN. If the lesions become infected, antibiotics or antibiotic ointment may be prescribed.[3] 

All treatments are typically used in combination with habit reversal therapy, which aims to reduce the frequency of scratching the skin. This can be helpful in slowing down or stopping the cycle of itching and scratching that is associated with PN. Some people wear gloves while they sleep to reduce scratching.[4] 

You can read further treatment information by visiting the American Osteopathic College of Dermatology (ACOD) information page on prurigo nodularis.[1]
Last updated: 4/19/2018

Prognosis Prognosis


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Although treatment options can help relieve itching and reduce the number of nodules, most people with prurigo nodularis (PN) do not have a complete resolution of the nodules even with treatment. The itching associated with the disease can be very intense, and it may affect the person’s ability to sleep at night or to enjoy everyday activities. This can result in increased stress and depression. It is important to speak with your doctor if you are having signs or symptoms of depression.[3][4]
Last updated: 4/19/2018

Find a Specialist Find a Specialist


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If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

  • The American Academy of Dermatology has an online tool that can help you find your nearest dermatologist. Click on the link above to access this tool.

Research Research


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Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Prurigo nodularis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations


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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

  • American Osteopathic College of Dermatology
    P.O. Box 7525
    Kirksville, MO 63501
    Toll-free: 800-449-2623
    Telephone: 660-665-2184
    Fax: 660-627-2623
    E-mail: dermatology@aocd.org
    Website: http://www.aocd.org/
  • Nodular Prurigo International
    136 Bedford Street South
    Liverpool, L7 7DB United Kingdom
    Telephone: +44 (0)151 709 1432
    E-mail: info@nodular-prurigo.org.uk
    Website: http://www.nodular-prurigo.org.uk/

Social Networking Websites

  • Visit the following Facebook groups related to Prurigo nodularis:
    Nodular Prurigo International
    Nodular Prurigo Awareness

Organizations Providing General Support

  • Skin Support
    Website: http://www.skinsupport.org.uk/
Do you know of an organization? We want to hear from you.

Learn More Learn More


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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

  • The British Association of Dermatologists has a patient information leaflet about Prurigo nodularis.
  • 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.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Prurigo nodularis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

  • Kowalski EH, Kneiber D, Valdebran M, Patel U, Amber KT. Treatment-resistant prurigo nodularis: challenges and solutions. Clin Cosmet Investig Dermatol. 2019 Feb 28;12:163-172.

    Wureshi AA et al., A Systematic Review of Evidence-Based Treatments for Prurigo Nodularis. Journal of the American Academy of Dermatology. March 2019;80(3):756-764.

    Mullins TB, Sharma P, Sonthalia S. Prurigo Nodularis. 2019 Jun 5. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.

News & Events News & Events


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News

  • Rare Disease Day at NIH 2021
    March 1, 2021

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Prurigo Nodularis. American Osteopathic College of Dermatology. http://www.aocd.org/page/PrurigoNodularis. Accessed 4/7/2018.
  2. Oakley A. Nodular prurigo. DermNet NZ. September 2014; https://www.dermnetnz.org/topics/nodular-prurigo/.
  3. Prak AH and De la Rosa KM. Prurigo Nodularis. Medscape Reference. June 5, 2017; https://emedicine.medscape.com/article/1088032-overview.
  4. Watsky K. Prurigo nodularis. UpToDate. March 8, 2018; https://www.uptodate.com/contents/prurigo-nodularis.
  5. Ossorio-García L, Jiménez-Gallo D, Rodríguez-Mateos ME, Arjona-Aguilera C & Linares-Barrios M. Treatment of prurigo nodularis with lenalidomide. Dermatol Ther. March 2017; 30(2):https://www.ncbi.nlm.nih.gov/pubmed/27891738.
  6. Lee HG, Stull C, and Yosipovitch G. Psychiatric disorders and pruritis. Clinics in Dermatology. May-June 2017; 35(3):273-280. https://www.ncbi.nlm.nih.gov/pubmed/28511824.
Do you know of a review article? We want to hear from you.
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