What is morphea?

Autoimmune diseases occur when the immune system, which normally protects you from infections and cancer, makes a mistake and starts to damage your body’s own tissues.   Morphea (syn. localized scleroderma) occurs as a result of autoimmune fibrosing (leather-like) injury to the skin and sometimes the underlying fat, muscle and bone.  Morphea is sometimes referred to as “localized scleroderma”.  However, despite their similar names, patients with morphea are not at increased risk of developing internal organ scleroderma involvement (systemic sclerosis).

In morphea, the immune system mistakenly causes inflammation of the skin.  This inflammation then causes fibrosis (hardening) of the skin and underlying tissues.  There are several different types of morphea some of which characteristically begin in adults while others characteristically start in childhood.

Adult-onset morphea (circumscribed morphea, generalized morphea).  Circumscribed morphea causes circular fibrosis of the skin and subcutaneous fat in one to three areas.  It is the most common subtype in adults and is unlikely to involve the underlying muscle or bone.  Generalized morphea presents as four or more areas of skin and subcutaneous fibrosis.  Patients with generalized morphea are more likely to feel tired and to have joint pain than other variants of morphea.  Adults can also get linear morphea (see below).

Juvenile-onset morphea (linear morphea, pansclerotic morphea).   Linear morphea occurs in a line usually on the head, arm or leg.  When involving the scalp and face, linear morphea has been referred to as morphea en coup d’sabre (linear scleroderma can produce a scarred appearance on the face and scalp similar to what might result from the strike of a sword).  Linear morphea is the most common subtype of morphea in children.  Linear morphea commonly affects the underlying muscle, bone, and if on the head the brain and is therefore treated aggressively.  Some authorities feel that linear morphea and progressive facial hemiatrophy (Parry Romberg syndrome) are related conditions.  Progressive facial hemiatrophy produces shrinkage of tissues beneath the skin, usually on only one side of the face.

Pansclerotic morphea is the rarest type of morphea.  Pansclerotic morphea results in fibrosis of the skin on the trunk and/or extremities.  In addition to causing fibrosis of the skin, pansclerotic morphea also results in fibrosis of the subcutaneous fat, underlying muscle and bone tissue.  Pansclerotic morphea can be localized to relatively small areas of the body or can involve large areas of the body.  Severe cases of pansclerotic morphea can produce long-term disability and on rare occasion have resulted in death.

Mixed variant morphea is a designation for patients who demonstrate a combination of linear and circumscribed morphea or linear and generalized morphea.

What caused my morphea?

The cause of morphea is unknown at this time.  We know that patients with family histories of autoimmune diseases are more likely to get morphea, so there is probably a genetic link.  Morphea has been reported to occur after injuries and radiation therapy, which may play a role as a disease trigger.

What other complications am I at risk for?

Patients with generalized morphea may experience fatigue and joint pain.  Patients with generalized and pansclerotic morphea may experience difficulty with deep breathing, if all of the skin on their chest is involved.  If a limb is involved, children with linear morphea may have shortening of the involved limb.  Linear morphea involving the scalp and face may develop seizures and neurologic problems.  Seizures and neurological deficits may also complicate progressive facial hemiatrophy.  Children with linear morphea of the head may also have eye involvement and should see an ophthalmologist.

What can I expect?

Every morphea patient is different and therefore predictions of disease course are difficult.  For the vast majority of patients with morphea, the condition will “burn out” with or without treatment in 3 to 5 years.  This means that no new lesions will develop, existing lesions will not grow, and any red or purple discoloration around the lesions will turn brown (postinflammatory hyperpigmentation).  The vast majority of patients are responsive to therapy — be it creams, light therapy or systemic immunosuppressive drugs such as methotrexate.  The subtype of morphea you have will determine the appropriate treatment options.  When the disease is in the burnt out phase it generally has a scarred appearance.  Some patients heal very well with their morphea lesions barely noticeable to others.  Some patients have obvious morphea lesions that are then stable over time.  Some patients develop another “active phase” after a damage phase during which they again develop new lesions and these lesions grow.

Typically, morphea does not change the length of your life span.  However, the rare severe forms of morphea discussed above can produce long-term disability and on extremely rare occasion, death.

What can I do to make my skin better or worse?

Morphea treatment depends on morphea subtype.  Most patients with circumscribed morphea will do very well with medicated creams.  Patients with generalized, linear, mixed and pansclerotic morphea may need light therapy or pills to suppress the inflammation.  Two main type of phototherapy have been found to be helpful for the itch in systemic sclerosis, narrow band UVB and UVA1.  Phototherapy generally requires 2 to 3 visits per week over the course of 2 to 3 months.  Pills that calm the immune system, like methotrexate, mycophenolate mofetil, prednisone, hydroxychloroquine, and other medications, may be used.

All patients will have less itching and symptoms if the morphea lesions are kept moisturized.  It is important to find a thick moisturizing cream that you are comfortable applying twice a day to decrease symptoms.  Lotions (products that can be poured or pumped) are mostly made up of water, which is not moisturizing enough for the skin.  Sun exposure may be helpful for morphea lesions, but do not over do it.  Ten to 15 minutes of exposure to midday sunlight two to three times per week may improve the morphea lesions.

Is my family at risk?

Morphea does not tend to run in families; however, if you have morphea your family has a slightly increased risk of having other autoimmune diseases such as vitiligo, psoriasis, lupus, and thyroiditis.

Other resources

Archives of Dermatology

Mayo Clinic Morphea Patient Information