What are the other Names for this Condition? (Also known as/Synonyms)

• Circumscribed Scleroderma
• Localized Scleroderma (Morphea)
• Morphoea

What is Morphea? (Definition/Background Information)

• Morphea is a localized type of Scleroderma that is characterized by thickened skin patches and plaques, which can increase and decrease in size. In some cases, the patches resolve without treatment
• Scleroderma is a chronic connective tissue disorder of unknown cause. Connective tissues give support to the organs and skin. Individuals with Scleroderma have thick or hard connective tissue

There is currently a debate about how Scleroderma should be classified. A majority believe that it should be classified as an autoimmune rheumatologic disorder. Due to this, many researchers believe that the damage is caused by immune cells mistakenly attacking one’s own healthy tissue.

Scleroderma can be classified into two broad categories, which include Localized Scleroderma and Systemic Scleroderma:

• Localized Scleroderma:
  • It often affects the skin tissues and does not cause harm to the other major organs
  • There are two types of Localized Scleroderma, namely Morphea and Localized Linear Scleroderma
• Systemic Scleroderma:
  • It affects the skin, tissue beneath the skin, major organs, and blood vessels
  • Systemic Scleroderma also has two different types, namely Limited Systemic Scleroderma and Diffuse Systemic Scleroderma

Morphea is a type of Localized Scleroderma of unknown cause.

• There are several different types of Morphea, which include Circumscribed Morphea, Generalized Morphea, and Superficial Morphea. The healthcare provider can assist in identifying the type of Morphea an individual is diagnosed with
• The signs and symptoms of Morphea do not progress in a rapid manner, but can vary greatly from individual to individual. Hardening and tightening of the skin in a patch formation and restricted movement of the affected skin may be observed
• The complications of Morphea include tightening of facial skin to a great degree causing the mouth to become narrower and smaller, in the rare case of facial involvement
• There is currently no cure for Morphea, but medications and therapy may be used to control symptoms and prevent complications
• The prognosis of Morphea is usually excellent, as it is a localized form of Scleroderma. Individuals with this form of Scleroderma do not have involvement of other organs other than the skin

Who gets Morphea? (Age and Sex Distribution)

• Individuals of any age can get Morphea; children can also be affected. A majority of Localized Scleroderma cases appear before age 40
• It is not uncommon for young children to have the signs and symptoms of Morphea, as it usually occurs at a younger age group compared to Systemic Scleroderma
• Like Systemic Scleroderma, it is thought that Morphea occurs more often in females
• Scleroderma is known to occur worldwide, in all races and ethnic groups. Localized Scleroderma is more common in individuals of European descent than African Americans

What are the Risk Factors for Morphea? (Predisposing Factors)

The risk factors for Morphea may include:

• Females are more likely than males to have Morphea
• Some studies have shown that individuals who have worked or been exposed to silica dust and polyvinyl chloride (PVC) may have an increased incidence

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Morphea? (Etiology)

The exact cause of Morphea is presently unknown. It is thought to occur due to genetics. Research is being currently performed to identify the relevant causal factors.

• Many researchers believe that Scleroderma is an autoimmune disorder, where the body’s immune system mistakenly attacks healthy tissues resulting in damage of the tissues and organs
• Some studies have revealed that there may be a higher risk for the condition, due to occupational exposure to chemicals such as silica dust and PVC

However, research has established the following on Morphea:

• It is not an infectious or contagious condition, which means that it cannot be passed on from one individual to another through contact. It is also not a malignant condition
• It is not a genetic disorder, which means it cannot be transmitted to the children of an individual diagnosed with Morphea

What are the Signs and Symptoms of Morphea?

The signs and symptoms of Morphea can vary greatly from individual to individual. This might create confusion in many, since one may not have all of the signs and symptoms described for the condition. In some cases, Morphea can be severe, causing significant signs, symptoms, and complications.

The signs and symptoms of Morphea can include:

• Hardening and tightening of the skin in a patch or plaque formation, which can be ovals or straight lines. This can cause restricted movement of the affected area of the skin
• Irregular pigmentation of skin
• Ulcer formation on the fingertips or toes

Morphea predominately affects the skin, but individuals can also experience the following symptoms:

• Joint pain and painful joint movement
• Muscle pain
• Fatigue
• Shivers
• Headaches

How is Morphea Diagnosed?

Morphea can be difficult to diagnose, as other conditions may have similar signs and symptoms. The presence of common symptoms or skin that thickens quickly makes it easier to diagnose Morphea. The following tests and procedures may be used to diagnose Morphea:

• A thorough physical examination along with a complete medical and family history
• Blood tests, such as anti-nuclear antibody panel, erythrocyte sedimentation rate, complete blood count, rheumatoid factor levels, antibody testing for other autoimmune disorders, and other tests
• Blood tests to rule out the possibility of internal systemic disease
• Kidney function tests including creatine level, BUN level, glomerular filtration rate (GFR), and urine analysis
• Chest X-ray and CT scan of the lungs to determine the extent of lung scarring (pulmonary fibrosis)
• Pulmonary function test (PFT)
• Electrocardiogram and echocardiogram to determine heart involvement, caused by heart scarring (cardiac fibrosis)
• Skin biopsy: Cells and tissues are examined by a pathologist under a microscope. Pathological examination is generally considered to be a ‘gold standard’ for a definitive diagnosis. The pathologist arrives at a diagnosis after analyzing pathology findings along with clinical information of the patient. A pathologist may perform special studies on tissue samples to aid in the final diagnosis

Even though Limited Scleroderma types have local signs and symptoms, the physician may request additional tests to rule-out systemic forms of Scleroderma.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Morphea?

Complications due to Morphea can range from mild to severe and could include:

• After resolution of the active phase of Morphea, when redness and firmness have resolved, brown patches of post-inflammatory hyperpigmentation can remain
• Spina bifida is found in over 30% of the individuals who are diagnosed with Morphea. In addition, other skin disorders may be present along with Morphea; these include Vitiligo, an autoimmune pigment loss condition, ichthyosis, thickening and scaling of the skin , and alopecia areata (autoimmune hair loss)
• Other systemic conditions may also be present with Morphea, such as primary biliary cirrhosis, dermatomyositis, pemphigus, and systemic lupus erythematosus (SLE)

How is Morphea Treated?

Localized Scleroderma can sometimes get better without treatment. The skin-related signs and symptoms of Morphea may fade on their own within 3-5 years.

The treatment options for Morphea include:

• Individual plaques  can be treated with topical corticosteroids, calcipotriene (a vitamin D cream), and calcineurin inhibitors
• Phototherapy, usually narrowband UVB or 309 nm excimer laser treatments
• Oral methotrexate is sometimes used for progressive cases
• Long-term oral antibiotics are sometimes used, not because Morphea is an infection, but for their anti-inflammatory side effects
• Occupational or physical therapists can help individuals with Morphea manage pain, increase their strength, and uphold their independence in their everyday lives

Treatment measures for painful joints with or without restricted movements include:

• Regular exercise and stretching that helps loosen up the joints
• Pain medication to reduce joint pain

Treatment measures for skin signs and symptoms include:

• Regular use of sunscreen, avoiding hot showers, and regular exercising
• For individuals living in a dry climate with low humidity, the use of a humidifier can help with the signs and symptoms
• Plastic surgery may be an option for cosmetic issues; a plastic surgeon can advise on the available treatment options
• It is always important to follow the physician’s advice and take the prescribed medication regularly
• A therapist or certain support groups may be consulted on the variety of social issues that can be caused by Scleroderma, which include self-esteem issues, depression, anxiety, and relationships with friends, family, and partners

How can Morphea be Prevented?

Currently, there are no specific methods or guidelines to prevent Morphea. However, through proper dietary and lifestyle changes, some of the signs and symptoms may be controlled.

What is the Prognosis of Morphea? (Outcomes/Resolutions)

• The prognosis of Morphea is usually excellent, as it is a localized form of Scleroderma. Individuals with this condition do not have involvement of other organs other than the skin
• However, the manner in which Morphea changes and progresses over time varies among individuals. Some individuals respond well to treatment and generally have a better prognosis than those who do not respond well to treatment

Additional and Relevant Useful Information for Morphea:

• Research is currently underway to identify and understand more about all forms of Scleroderma, localized and systemic . Studies are being completed to understand the genes that may be involved with the development of the condition and to find medicines to completely treat Morphea
• Scleroderma used to be called progressive systemic sclerosis, but this term is no longer used since Scleroderma does not always progress by nature. This means that the signs and symptoms do not progress with equal severity in all individuals