It is more common in females than in males.
Reproduced with permission from DermNet New Zealand and Raimo Suhonenwww.dermnetnz.org2023.
Types of Morphea
Morphea can occur in distinct patterns of symptoms.
Reproduced with permission from ©DermNet New Zealand and © Raimo Suhonenwww.dermnetnz.org2023.
These have been categorized in several different ways, so the terminology can get pretty confusing.
Morphea vs. Morphea is related to a disease called systemic sclerosis, which also sometimes just goes by the namescleroderma.
But morphea can be thought of as one pop in of scleroderma.
Morphea and systemic sclerosis have some similarities in their symptoms and underlying causes.
They both involve underlying inflammation that leads to scarring and thickening of the skin.
However, even though they are both kinds of scleroderma, morphea and systemic sclerosis are quite different.
Systemic sclerosis can affect internal organs (such as the lungs, esophagus, and heart).
It is typically a much more serious condition than morphea, which doesnt ever have this involvement.
Reassuringly, even though morphea and systemic sclerosis share some similarities, they are not the same disease.
Only very rarely do people with morphea go on to develop systemic sclerosis.
However, rheumatologists have been moving more towards using the term more generally to refer toall typesof localized scleroderma.
It doesnt affect the organs as extensively as diffuse systemic sclerosis (another punch in of systemic sclerosis).
However,limitedcutaneous systemic sclerosis is not the same disease as morphea (localizedscleroderma).
This cycle will often stop on its own within a few years, even without intervention.
Linear morphea, however, tends to cause more long-lasting problems.
Most commonly, morphea doesnt cause issues other than those due to the physical appearance of the skin.
Occasionally these areas are itchy, but they usually arent painful.
Less commonly, other symptoms occur due to the presence of morphea in deeper layers of the body.
The area may be swollen.
Over time, the area may develop a whitish center.
As the inflammation dies down, the area may become thinner and tighter, leaving a darker appearance.
Sometimes linear morphea just affects the outermost layer of skin, theepidermis.
But sometimes linear extends into the deeper skin (dermis) and even deeper into a layer calledfascia.
Sometimes it even extends into the muscles and joints.
This is called linear morphea en coup de sabre, French for the blow of a sword.
When linear morphea affects the entire side of the face, this is sometimes called progressive facial hemiatrophy.
This is sometimes called generalized morphea.
When people have symptoms both of linear and circumscribed morphea, that sometimes goes by the term mixed morphea.
This is sometimes called deep morphea or pansclerotic morphea.
Depending on location, these may also cause additional problems, like joint contractures and deformities.
In another rare form of morphea, bullous morphea, blisters also occur on top of these skin changes.
Causes
We arent entirely clear on what causes morphea.
However, morphea seems to be a kind of autoimmune disease.
In this case, dysregulation of part of the immune system drives the symptoms.
For example, certain inflammatory cytokines (immune signaling molecules) can be overproduced.
Certain immune cells likeB cells and T cellsmay become unnaturally activated.
This fibrosis accounts for the rigid, tense areas seen in morphea.
This fibrosis is part of why affected areas never get completely back to normal.
Even after the initial inflammation has subsided, some scar-like tissue remains.
The specific symptoms will depend on how deeply this scar tissue extends into the body.
Most commonly this fibrosis only extends to the epidermis.
But in some people the problem goes into deeper layers.
Other factors may also play a role in triggering the disease in some people.
Some of these might include:
Morphea is not an infectious disease.
Its not contagious, so you cant spread it to someone else.
It is also not a sign of cancer.
Diagnosis
The medical history and medical exam are key parts of diagnosis.
Sometimes these alone will make your clinician strongly suspect the diagnosis.
Your medical provider will ask about your recent symptoms, your medications, and your past medical problems.
A medical exam with an emphasis on skin issues also gives important clues.
A skin biopsy may also be important to rule out other possibilities and confirm a diagnosis.
Your healthcare provider may order some basic blood work as part of the diagnosis.
However, standard blood tests like acomplete blood count (CBC)usually arent that helpful in diagnosing morphea.
However, researchers are still working to understand how helpful these tests might be for someone with morphea.
Sometimes imaging tests are needed to evaluate morphea.
For example,magnetic resonance imaging (MRI)might be needed for morphea affecting their head and face.
It can also be used in people who have morphea that has affected layers deeper than the epidermis.
A generalist physician can diagnose morphea, but the expertise of a dermatologist or rheumatologist is sometimes helpful.
Specific treatment will depend on the bang out and severity of morphea involved.
People who have circumscribed morphea that just affects the outer layer of skin are usually prescribed ointments or creams.
Topical steroidsare often the first choice.
Phototherapyis also an option for these people.
UVA or narrowband UVB can be used.
Oral medications that affect the immune system might be needed as well, particularly if phototherapy isnt an option.
For example, oral steroids are often given along with methotrexate.
Other medications affecting the immune system might also be used, such as CellCept (mycophenolate mofetil).
Other potential treatments for morphea are currently under investigation, but we dont know as much about their use.
These therapies target the active, inflammatory phase of the disease.
As another example, surgery might be necessary for someone with deformations from morphea en coup de sabre.
Coping
Morphea can have a significant psychological impact.
Professional counseling can be very helpful for people trying to navigate the disease.
Even people with more mild disease might be self-conscious about cosmetic changes from the condition.
By working closely with your clinician, it’s possible for you to minimize the impact on your life.
2013;2013:467808. doi:10.1155/2013/467808
Hickman RJ.Study probes new gene therapy for severe, localized scleroderma.The Rheumatologist.
American College of Rheumatology.
August 16, 2019.
2013;104(8):654-66. doi:10.1016/j.adengl.2012.10.012
