Rare Pediatrics News

Disease Profile

Morphea

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

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ICD-10

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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

Skin Diseases

Summary

Morphea is a skin condition that causes patches of reddish skin that thicken into firm, oval-shaped areas. It is a form of scleroderma. Patches most often occur on the abdomen, stomach, and back, and sometimes on the face, arms and legs.[1][2] Morphea is classified according to the localization of the lesions and the depth of tissue involvement into localized or circumscribed (limited to one or several patches) , generalized (spread over larger areas of the body), linear (with linear lesions involving the outer skin and deeper layers), and pansclerotic (that involves not just the outer skin but the deeper layers, and sometimes the bone underneath, and also restricts movement of the joints).[2][3][4] The underlying cause of morphea is unknown. It may be associated with an abnormal immune response, or be triggered by radiation therapy, repeated trauma to the affected area, or a recent infection.[2] It usually goes away without treatment within 3 to 5 years, but some people are left with darkened areas of skin or rarely muscle weakness.[1] Treatment may be used to control symptoms until it resolves, and may include phototherapy, prescription vitamin D creams, or corticosteroids.[2]

Symptoms

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.[2] 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.[1][2] According to the localization and deepness of the lesions morphea may be classified in:[1][2][4]

  • 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.

Cause

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.[4]

Treatment

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.[5]

Detailed information about how morphea might be treated can be viewed here on Medscape Reference's website.

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

    • Visit the Morphea group on Facebook.

      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 Mayo Clinic Web site provides further information on Morphea.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.
      • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

        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.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Morphea. Click on the link to view a sample search on this topic.

          Selected Full-Text Journal Articles

            References

            1. Handout on Health: Scleroderma. NIAMS. February, 2015; https://www.niams.nih.gov/Health_Info/Scleroderma/default.asp#2.
            2. Morphea. Mayo Clinic. September 29, 2015; https://www.mayoclinic.org/diseases-conditions/morphea/basics/definition/con-20028397.
            3. Nguyen JV. Morphea. Medscape Reference. 2016; https://emedicine.medscape.com/article/1065782-overview.
            4. Heidi Jacobe. Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults. UpToDate. Waltham, MA: UpToDate; September, 2016;
            5. Heidi Jacobe. Treatment of morphea (localized scleroderma) in adults. UpToDate. Waltham, MA: UpToDate; September, 2016;
            6. Nguyen JV. Morphea. Medscape Reference. 2017; https://emedicine.medscape.com/article/1065782.
            7. Oakley A. Morphea. DermNet NZ. 1999; https://www.dermnetnz.org/topics/morphoea/.

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