What Is It, Causes, Treatment, and More
Author: Ali Syed, PharmD
Editors: Ahaana Singh, Józia McGowan, DO, FACOI, FNAOME, CS
Illustrator: Abbey Richard
Copyeditor: Joy Mapes
What is morphea scleroderma?
Morphea scleroderma, more commonly known as morphea, is a skin condition characterized by discolored and thickened patches of skin on various areas of the body. It is a form of localized scleroderma, which is an inflammatory condition that causes hard and thickened patches of skin on different areas of the body, with no involvement of internal organs.Morphea scleroderma is classified according to the shape and location of the patches, the amount of skin affected, and the depth of tissue involvement. The various classifications of morphea include circumscribed morphea, generalized morphea, and linear morphea.
Is morphea scleroderma an autoimmune disease?
Morphea scleroderma is often considered an autoimmune disease, but this theory remains unconfirmed. Autoimmune diseases occur when the immune system, which normally protects us from infection, mistakenly attacks healthy body tissues. Individuals with morphea may have certain markers in their blood called autoantibodies. Autoantibodies are often involved in autoimmune disease, which supports the theory that morphea is an autoimmune disease.
What causes morphea scleroderma?
While the exact cause of morphea is unknown, potential contributing factors include an atypical reaction of the immune system, recent infection, repeated skin trauma, exposure to chemical toxins, or exposure to radiation. Morphea may also be linked to an overproduction of collagen, which is a protein in the skin that provides structural support. Excessive collagen deposition may lead to parts of the skin becoming stiff and hard.Generally, individuals with a family history of morphea or autoimmune diseases, White females, and children under the age of 18 may be at increased risk of morphea scleroderma.
How rare is morphea scleroderma?
What are the signs and symptoms of morphea scleroderma?
In general, signs of morphea include the development of red- or purple-colored oval patches on the skin, which usually thicken and become white over time. Morphea is typically asymptomatic, but some individuals may experience itching or pain. The exact signs and symptoms of morphea scleroderma can vary depending on the specific classification. Complications of morphea scleroderma may cause significant, irreversible cosmetic and functional impairment.
Circumscribed morphea is the most common subtype that predominates in adults, characterized by one or a few (i.e., 3 to 4) oval-shaped, thickened patches of skin, which are usually painless but may be itchy. The patches vary in pigment, with some areas appearing darker or lighter than the surrounding skin. Often, a violet-colored border may be seen around the patches when they are still actively growing.
Meanwhile, generalized morphea, which also primarily affects adults, is characterized by larger skin patches than in cases of circumscribed morphea. These patches tend to cover more body surface, as well as deeper tissues. Due to the extensive body surface involved, individuals with generalized morphea may develop disfigurement and joint complications, leading to joint pain and limited joint function.
A third subtype of morphea scleroderma, linear morphea, typically presents in children. It frequently appears as a linear patch of thickened, discolored skin on the arms or legs, but patches may also appear on an individual's forehead. These thickened patches may extend deep into tissues; affect muscles, joints and bones; and result in deformities. Linear morphea may lead to serious problems in children, such as delayed growth or eye involvement.
How is morphea scleroderma diagnosed?
Morphea scleroderma is commonly diagnosed after careful examination of the skin and a thorough evaluation of an individual's signs, symptoms, and family history. Further tests may be conducted to support diagnosis, such as blood tests to investigate for autoimmune diseases or a confirmatory skin biopsy, in which a small sample of the affected skin is removed and tested.
How is morphea scleroderma treated?
Morphea scleroderma typically resolves on its own without treatment within a few years, though some individuals may be left with darkened areas of skin. Until morphea resolves, treatment usually focuses on controlling symptoms. Treatment varies depending on the classification and severity of the morphea, but it tends to be more effective when started soon after the patches appear. For mild cases of morphea, treatment is optional.
Some treatment options for morphea treat only the outer layers of the skin. These include topical corticosteroids, used to reduce itching and inflammation of the skin, and prescription vitamin D creams, which may soften the patches and decrease the growth and formation of new ones. For more severe types of morphea, deeper layers of the skin may be treated by exposing the skin to artificial ultraviolet light in a process called phototherapy. Depending on the severity of morphea, oral medications that suppress the immune system, such as oral corticosteroids and methotrexate, may also be considered.
Lifestyle modifications to control signs and symptoms of morphea scleroderma may include applying moisturizers to soften the skin and avoiding hot showers, harsh soaps, and fragrances that can dry out the skin. Application of sunscreen before going outdoors may help prevent further skin damage.
What are the most important facts to know about morphea scleroderma?
Morphea scleroderma is a skin condition characterized by hardened, discolored, and thickened patches of skin on various areas of the body without internal organ involvement. The exact cause of morphea is unknown. However, it is considered an autoimmune disease and is associated with various predisposing factors. Signs and symptoms of morphea scleroderma largely depend on the presenting subtype or classification, three of which are circumscribed morphea, generalized morphea, and linear morphea. Morphea scleroderma is commonly diagnosed after careful examination of the skin and a thorough evaluation of an individual's signs, symptoms, and family history. Blood tests and skin biopsy may also assist with diagnosis. Morphea scleroderma may resolve on its own without treatment within a few years, and treatment is often optional, depending on the classification and severity of morphea.
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Resources for research and reference
Christen-Zaech, S., Hakim, M., Afsar, F., & Paller, A. (2008). Pediatric morphea (localized scleroderma): Review of 136 patients. Journal of the American Academy of Dermatology, 59(3): 385-396. DOI: 10.1016/j.jaad.2008.05.005
Fett, N. (2013). Morphea (localized scleroderma). JAMA Dermatology, 149(9): 1124. DOI: 10.1001/jamadermatol.2013.5079
Fett, N., & Werth, V. (2011). Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis. Journal of the American Academy of Dermatology, 64(2), 217-228. DOI: 10.1016/j.jaad.2010.05.045
Johns Hopkins Medicine. (n.d.). Types of scleroderma. In Health: Conditions and diseases. Retrieved February 2, 2021, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/scleroderma/types-of-scleroderma
National Center for Advancing Translational Sciences. (2018). Morphea. In Genetic and Rare Diseases Information Center (GARD). Retrieved February 1, 2021, from https://rarediseases.info.nih.gov/diseases/10485/morphea
Saracino, A. (2017). Morphea. In DermNet NZ. Retrieved February 1, 2021, from http://www.dermnetnz.org/topics/morphoea/
Peterson, L., Nelson, A., & Su, W. (1995). Classification of morphea (localized scleroderma). Mayo Clinic Proceedings, 70(11): 1068-1076. DOI: 10.4065/70.11.1068Morphea information for patients. (n.d.). In Rheumatologic Dermatology Society: For patients. Retrieved February 2, 2021 from, https://www.rheumaderm-society.org/morphea-information-for-patients/