National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Localized scleroderma



I have been diagnosed with morphea. Can you please provide me with patient-friendly information about this disease?


What is localized scleroderma?

Localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. Collagen is a protein normally present in our skin that provides structural support. However, when too much collagen is made, the skin becomes stiff and hard.[1] Localized types of scleroderma are those limited to the skin and related tissues and, in some cases, the muscle below. Internal organs are not affected by localized scleroderma, and localized scleroderma can never progress to the systemic form of the disease. Often, localized conditions improve or go away on their own over time, but the skin changes and damage that occur when the disease is active can be permanent. For some people, localized scleroderma is serious and disabling.[2]

There are two generally recognized types of localized scleroderma: morphea and linear.[2]

 

Last updated: 4/3/2012

What causes morphea?

The underlying cause of morphea is poorly understood. Theories on the cause are often drawn from studies of systemic sclerosis. A variety of factors, including autoimmunity, genetics, and vascular dysfunction may play a role in morphea. Multiple environmental factors (such as radiation, infections, skin trauma, or environmental exposures) also have been proposed as contributors to the development of morphea.[3]
Last updated: 2/15/2018

What are the signs and symptoms of morphea?

The signs and symptoms of morphea can vary depending on the type and severity. Morphea usually causes reddish patches of skin that thicken (sclerosis) into firm, oval-shaped areas. The center of each patch may develop a lighter center.[4] These patches sweat less than unaffected areas and may lose hair over time. Patches most often occur on the chest, stomach, and back, and sometimes occur on the face, arms, and legs.[5][4] According to the localization and deepness of the lesions morphea may be classified in:[5][4][3]
  • Localized or circumscribed which is limited to one or several patches on the trunk, legs and arms 
  • Generalized, spread over larger areas of the body, in the trunk, legs and limbs 
  • Linear,  where there are linear bands of thick skin that involve the deeper layers of the skin and are localized in the legs, arms, upper body or head
  • Morphea profunda or pansclerotic, which involves also the tissue below the skin and may restrict the joint movement.
Last updated: 2/15/2018

How might morphea be treated?

Treatment for morphea is aimed at controlling the symptoms and depends on the severity and body surface area involved. In general, active disease (present <3 months or with symptoms of inflammation) is most responsive to treatment. Because morphea typically goes away on its own eventually, treating the condition is optional for uncomplicated, localized cases.

The majority of adults with morphea are managed with observation, topical medications, or phototherapy. Aggressive, systemic therapy with methotrexate and/or glucocorticoids is needed for those with active, deep morphea; lesions that may cause significant cosmetic disfigurement; joint contractures; or other functional impairment. Occupational and physical therapy are also important for those at risks for physical limitations. Phototherapy is also preferred for those with generalized morphea, but it is not an option for those with deep morphea due to the poor ability of ultraviolet light to penetrate deep tissues.[6]

Detailed information about how morphea might be treated can be viewed here on Medscape Reference's website.
Last updated: 2/15/2018

What is the long-term outlook for people with morphea?

Morphea usually has a benign, self-limited course and life expectancy is typically normal. When lesions are superficial and circumscribed, the skin condition tends to slowly go away with time, with each individual lesion lasting an average of 3-5 years. However, some patients develop new lesions over their lifetimes.[7] Even after morphea resolves, changes in skin color may last for years.[8]

Linear morphea usually lasts longer, but also eventually improves.[8] Linear and deep (panslerotic) morphea may cause considerable morbidity, especially in children, interfering with growth. Joint contractures, limb-length discrepancy, and prominent facial atrophy (shrinkage of underlying tissues) can cause substantial disability and deformity in people with linear or deep morphea.[7] Limbs affected by severe morphea may be stiff and weak if there is muscle wasting.[8]
Last updated: 2/15/2018

Who can I contact to learn more about morphea and other forms of localized scleroderma?

You can learn more about morphea and other forms of localized scleroderma by visiting the following link from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), part of NIH.
http://www.niams.nih.gov/hi/topics/scleroderma/scleroderma.htm#2

Additionally, NIAMS offers printed materials on scleroderma. We recommend calling the toll-free number below to request a copy of this information.

National Institute of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
National Institutes of Health
1 AMS Circle
Bethesda, Maryland 20892-3675
Toll free: 877-22-NIAMS
Telephone: 301-495-4484
TTY: 301-565-2966
Fax: 301-718-6366
Email: niamsinfo@mail.nih.gov
Web site: http://www.niams.nih.gov/hi/index.htm

More information on scleroderma can be found at the following link from MEDLINEplus, the National Library of Medicine Web site designed to help you research your health questions.
http://www.nlm.nih.gov/medlineplus/scleroderma.html
Last updated: 9/12/2013

How can I locate research for morphea?

ClinicalTrials.gov lists trials that are studying or have studied morphea. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if you are eligible for any clinical trials.  

Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Email: prpl@mail.cc.nih.gov
Web site:  http://clinicalcenter.nih.gov/

If you are interested in enrolling in a clinical trial, you can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.
http://clinicaltrials.gov/ct2/info/understand

A tutorial about clinical trials that can also help answer your questions can be found at the following link from the National Library of Medicine:
http://www.nlm.nih.gov/medlineplus/tutorials/cancerclinicaltrials/htm/lesson.htm

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases (ORD), part of the National Institutes of Health.
http://rarediseases.info.nih.gov/Resources.aspx?PageID=8

Last updated: 6/23/2011

How can I find journal articles on morphea?

PubMed lists journal articles that discuss morphea. Click on the link to go to PubMed and review citations to these articles.
Last updated: 9/12/2013

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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  1. Localized Scleroderma. Scleroderma Foundation. http://www.scleroderma.org/site/DocServer/Localized.pdf?docID=317. Accessed 6/23/2011.
  2. . Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). May 2010; http://www.niams.nih.gov/Health_Info/Scleroderma/. Accessed 6/23/2011.
  3. Heidi Jacobe. Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults. UpToDate. Waltham, MA: UpToDate; September, 2016;
  4. Morphea. Mayo Clinic. September 29, 2015; http://www.mayoclinic.org/diseases-conditions/morphea/basics/definition/con-20028397.
  5. Handout on Health: Scleroderma. NIAMS. February, 2015; http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp#2.
  6. Heidi Jacobe. Treatment of morphea (localized scleroderma) in adults. UpToDate. Waltham, MA: UpToDate; September, 2016;
  7. Nguyen JV. Morphea. Medscape Reference. 2017; http://emedicine.medscape.com/article/1065782.
  8. Oakley A. Morphea. DermNet NZ. 1999; http://www.dermnetnz.org/topics/morphoea/.