Morphea: Evaluation and Extra

Morphea, also sometimes called “localized scleroderma,” is a rare autoimmune disorder that causes skin thickening, hardening, and color changes. It often only affects the outer layer of skin but sometimes leads to more serious issues due to the involvement of deeper parts of the body.

The disease most commonly begins to affect people in their fourth or fifth decade of life. However, it sometimes affects children as well. It is more common in females than in males.

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Types of Morphea

Morphea can occur in distinct patterns of symptoms. These have been categorized in several different ways, so the terminology can get pretty confusing. The two most common forms of morphea are:

  • Circumscribed morphea (also known as plaque morphea): Most common subtype in adults (and overall)
  • Linear morphea (sometimes called linear scleroderma): Most common subtype in children

However, clinicians have also used other descriptions for specific manifestations of morphea. Other forms of morphea you might hear mentioned are generalized morphea, bullous morphea, guttate morphea, pansclerotic morphea, deep morphea, and mixed morphea.

Morphea vs. Systemic Sclerosis (Scleroderma)

Confusingly, morphea is also sometimes called localized scleroderma, particularly in some places outside the United States. Morphea is related to a disease called systemic sclerosis, which also sometimes just goes by the name scleroderma. But morphea can be thought of as one type 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. (“Sclero” comes from the Greek word for hard, and “derma” from the word for 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 doesn’t 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.

Another point of confusion is that, in the past, morphea has sometimes been used to refer just to the circumscribed (plaque) type of morphea. However, rheumatologists have been moving more towards using the term more generally to refer to all types of localized scleroderma.

As if that weren’t complicated enough, there is a type of systemic sclerosis that goes by the name “limited cutaneous systemic sclerosis” or “limited scleroderma.” It doesn’t affect the organs as extensively as diffuse systemic sclerosis (another type of systemic sclerosis).

However, limited cutaneous systemic sclerosis is not the same disease as morphea (localized scleroderma). 

Symptoms

Symptoms of morphea tend to grow through periods of improving and worsening over time as new lesions form. An initial inflammatory period in an area is followed by a burnout phase with no active inflammation but with some residual permanent changes to the skin (and deeper tissues, if applicable). 

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 doesn’t cause issues other than those due to the physical appearance of the skin. Occasionally these areas are itchy, but they usually aren’t painful. Less commonly, other symptoms occur due to the presence of morphea in deeper layers of the body.

Circumscribed Morphea (Plaque Morphea)

One or two round-shaped areas are typically found on the trunk or limbs. They may first start to turn a reddish or purplish color and then start to turn hard, shiny, and thick. 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. Once this has happened, the skin is unlikely to ever return to its former appearance, although it usually somewhat softens over time.

Linear Morphea

In linear morphea, you don’t see round areas but rather linear streaks of hardened tissue. This usually happens just on one side of the body, most commonly on the extremities, face, or scalp.

Sometimes linear morphea just affects the outermost layer of skin, the epidermis. But sometimes linear extends into the deeper skin (dermis) and even deeper into a layer called fascia. Sometimes it even extends into the muscles and joints. Because of this, it can cause some additional health problems, like the following:

  • Limiting joint movement (if the morphea connects with a joint)
  • Muscle contractures (tightening of the muscles and joints that can cause deformity and pain)
  • Differences in limb length (from constricted growth) and changes in gait
  • Arthritis and muscle aches

There is also a dangerous form of linear morphea in which areas of the head are affected, causing hair loss and a depressed groove. This is called “linear morphea en coup de sabre,” French for “the blow of a sword.” Depending on where this groove is, it can cause serious issues, such as seizures and problems with the teeth, jaw, nose, lips, and eyes.

When linear morphea affects the entire side of the face, this is sometimes called progressive facial hemiatrophy.

Symptoms of Other Forms of Morphea

Sometimes areas of oval skin lesions affect not just one or two places but multiple parts of the body. This is sometimes called generalized morphea. When people have symptoms both of linear and circumscribed morphea, that sometimes goes by the term “mixed morphea.”

Some people with oval-plaque like lesions have areas that attach more deeply and not just to the outer layer of skin. 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. In guttate morphea, a person might have small white lesions with a similar appearance to another disease, lichen sclerosis.

Distinguishing Morphea from Systemic Sclerosis

People with morphea do not have any of the following:

  • Raynaud’s phenomenon (painful cold fingers in response to stress or cold)
  • Red, puffy hands
  • Tightening of the skin of the fingers (sclerodactyly)
  • Changes in the blood vessels of the nail

These symptoms likely indicate that a person has systemic sclerosis (scleroderma) instead.

Causes

We aren’t 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 like B cells and T cells may become unnaturally activated. The area gets inflamed, and certain cells help trigger fibrosis—the secretion of certain compounds usually used to make scar tissue.

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:

  • Having variations of certain genes (particularly ones important for the immune system)
  • Underlying damage to blood vessels in the area
  • Certain kinds of infections
  • Previous trauma to the area (e.g., through radiation exposure)

Morphea is not an infectious disease. It’s not contagious, so you can’t 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. Importantly, you’ll also be asked about whether you have symptoms that might correspond better to other medical conditions, like fever or Raynaud’s phenomenon.

A medical exam with an emphasis on skin issues also gives important clues. A thorough examination of the musculoskeletal system is also important, especially for people who seem to have a morphea subtype that involves deeper layers of tissue.

A skin biopsy may also be important to rule out other possibilities and confirm a diagnosis. Your doctor may order some basic blood work as part of the diagnosis. However, standard blood tests like a complete blood count (CBC) usually aren’t that helpful in diagnosing morphea.

You might get blood tests for certain autoantibodies, as some people with morphea are positive for some antibodies found in other types of autoimmune disease, like lupus and rheumatoid arthritis. 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.

Treatment

Unfortunately, we don’t have a cure for morphea, but a variety of treatments can help lessen symptoms. Specific treatment will depend on the type and severity of morphea involved. But we are still learning a lot about the best treatments to use in people with different kinds and degrees of morphea. 

People who have circumscribed morphea that just affects the outer layer of skin are usually prescribed ointments or creams. This is also true for people with linear morphea who only have skin symptoms. Such creams affect the immune response and modulate inflammation. 

Topical steroids are often the first choice. However, there are also topical creams that might be used, such as:

  • Protopic (tacrolimus)
  • Vitamin D cream such as Donovex (calcipotriol)
  • Zyclara (imiquimod)

In many people, such treatment can diminish symptoms of the disease, such as itching.

Phototherapy is also an option for these people. UVA or narrowband UVB can be used. This may include using a medication called psoralen (either taken orally or applied to the skin through a bath). Then the person goes through multiple phototherapy sessions, usually multiple times per week for several weeks.

Phototherapy is also a good option for people with widespread skin symptoms or morphea affecting the deeper layers of the body. Oral medications that affect the immune system might be needed as well, particularly if phototherapy isn’t 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 don’t know as much about their use. For example, researchers are exploring the following as other treatment options for morphea: 

These are all therapies sometimes used in other autoimmune conditions. Some clinicians may opt to try them, particularly if a person isn’t having a good response from other treatments or if they have too many side effects from steroid therapy.

These therapies target the active, inflammatory phase of the disease. It’s important to start treatment as soon as possible to prevent long-term damage that can’t be corrected after periods of active disease.

Physical therapy and occupational therapy can be key for people who have joint contractures or deeper muscle and joint involvement. For some people, certain procedures or surgical options may give them the best opportunity for cosmetic correction and full functionality. 

For example, a fat filler procedure might help someone with linear morphea achieve a more symmetrical appearance of their legs. As another example, surgery might be necessary for someone with deformations from “morphea en coup de sabre”.

Typically, surgery isn’t recommended in the initial inflammatory part of the disease but only after the condition is medically under control.

Coping

Morphea can have a significant psychological impact. Not surprisingly, people with more severe types of morphea tend to experience more problems related to this, and those with disfiguring disease may be most strongly affected. Professional counseling can be very helpful for people trying to navigate the disease.

A Word From Verywell

Especially for people with more severe forms of the disease, morphea can significantly impact quality of life. Even people with more mild disease might be self-conscious about cosmetic changes from the condition. By working closely with your clinician, you can minimize the impact on your life. 

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