Morphea

What is Morphea?

Morphea, also known as localized scleroderma, is a rare skin condition that causes inflammation and hardening of the skin and the layer just beneath it. It occurs in both children and adults. Morphea comes in many different forms. Some people only have small areas of skin affected with little discomfort, while others may have more serious forms that affect large areas.

Typically, Morphea affects only the skin and the layers just beneath it. However, in about 22% of patients, it can affect other parts of the body as well. In rare cases, this disease can cause problematic skin lesions that lead to stiff joints, abnormal limb growth, and other issues in children.

Because Morphea can look very different in different people, it can be hard to classify. The most recognized classification was proposed by Laxer and Zulian. In their system, Morphea can be categorized into a few main types: circumscribed morphea, linear morphea, generalized morphea, pan-sclerotic, and a mixed variety. Circumscribed morphea, which either affects the skin’s surface or deeper layers, is the most common type accounting for 60-65% of the patients. Linear morphea is named for the specific location of the lesion, either being on a limb or head.

In 2017, the European dermatology forum (EDF) came up with a newer classification. They identify the disease as: generalized type, limited type, linear type, deep type, and mixed type.

What Causes Morphea?

The cause of morphea, a skin condition that causes hard, discolored patches on the skin, is still largely unknown. However, experts believe that it may be linked to genetic factors, issues with the immune system, and exposure to certain environmental factors.

Strong associations have been found between morphea and two specific genetic markers, known as DRB1*04:04 in the HLA class II allele, and HLA-B*37 in the class I allele. In simpler terms, if you have these particular markers in your DNA, you might be more likely to develop morphea.

Additionally, the presence of certain auto-antibodies (proteins that the immune system mistakenly produces to target its own cells) could suggest that the immune system is not functioning properly. These include the antinuclear antibody (ANA), anti-single-stranded DNA (SS DNA), and anti-histone antibodies. These irregularities in the immune system could also play a role in the development of morphea.

Another hint at the role of the immune system is that family members of people with morphea seem to get other autoimmune diseases more frequently. Autoimmune diseases happen when your immune system mistakenly attacks your body.

Lastly, environmental factors, like physical injury, exposure to radiation, and friction, might also contribute to the development of morphea. Radiation, for instance, can cause the body to release molecules like interleukin 4 and 5. These molecules can trigger the release of another molecule, TGF-B, which induces the formation of fibrous connective tissues — a hallmark of morphea.

Risk Factors and Frequency for Morphea

Morphea is an uncommon condition that affects the skin. Women tend to get it more often than men. Normally, it affects women in their 40s. Despite this, it can also affect children, usually between the ages of 7 and 11.

As for prevalence, it ranges from 3.4 to 27 cases per 100,000 people. However, in the United States, the rate is lower, with 2 to 3 cases per 100,000 people. It’s also worth mentioning that white people are more likely to get Morphea than African Americans.

Morphea is a rare skin disease.
It affects women more often than men, particularly in their 40s.
It can also affect children, especially between ages 7 and 11.
The prevalence varies, but ranges from 3.4 to 27 cases per 100,000 people.
In the United States, there are 2 to 3 cases per 100,000 people.
Morphea is more common in white people than in African Americans.

Signs and Symptoms of Morphea

Morphea is a skin condition that appears in different types, with varying symptoms for each. In the early stages, morphea shows up as red skin patches which can be itchy and tender. Over time, these patches become hard with a purple border, leading to changes in skin color and some shrinking of skin and the fatty layer underneath. While most people see their symptoms go away after a few years, some continue to experience new patches and persistent symptoms.

Circumscribed morphea – This type appears as separated round or oval spots typically on the chest or upper body. The ‘superficial’ version affects only the outer layer of skin, while the ‘deep’ form extends into deeper layers and can scar. The deep form can sometimes cause issues like limited joint movement.
Linear morphea – This subtype is characterized by band-like patches, typically on the limbs or around the neck and head. It’s the most common in children and can affect deeper tissues, potentially leading to muscle weakness, joint limitations, and limb deformities.
En coup de sabre morphea – This kind results in a swooping patch on the forehead, extending to the scalp and causing hair loss. If the deeper layers of skin are involved, it can also cause eye and neurological symptoms like headache and seizures. In rare cases, it can lead to one-sided facial shrinkage.
Generalized morphea – This severe form presents as many patches or merged plaques and is more common in women. The patches can be deep and affect joint movements. It’s defined by the presence of more than four patches in at least two different body parts.

There are also very rare forms like bullous and guttate morphea. Other similar conditions include eosinophilic fasciitis and Parry Robertson syndrome. The term ‘mixed morphea’ is used when more than one subtype of morphea is present simultaneously.

Testing for Morphea

Morphea, a skin condition, is usually diagnosed by your doctor through a simple physical checkup. However, in certain cases, a skin biopsy might be necessary. This means taking a small sample of the skin, including the layer of fat beneath it, which will be analysed to confirm the diagnosis.

To understand morphea better, there are no specific antibodies linked to this condition, which are proteins our bodies produce to fight off harmful substances. However, some patients have tested positive for certain antibodies such as ANA, anti-histone Ab, and SS DNA Ab. A study showed that 30% of people with morphea had a positive ANA, compared to 11% without the condition. Additionally, a blood test that shows an unusually high number of a certain type of white blood cell (eosinophilia) and markers indicating inflammation might aid in diagnosing the condition. It’s important to note that if a person with morphea tests positive for ANA and also has symptoms of Raynaud’s disease (a condition that affects blood circulation), a screening for a condition called systemic sclerosis may need to be done.

When dealing with severe cases of morphea, using Magnetic Resonance Imaging (MRI) can provide a clearer understanding of how deep the skin lesions go and if there are any complications, such as thickening of the connective tissue beneath the skin, inflammation of the tissue that lines your joints, or inflammation of the muscles. Ultrasound, another imaging method, can also be a good option for routinely monitoring the disease.

Treatment Options for Morphea

When treating morphea, a skin condition that causes thick, hard skin patches, healthcare professionals need to consider the specific kind of morphea, how deep the condition has affected the skin, and how active the disease is. These factors are important in deciding the best treatment. Spotting and starting treatment early is vital to reduce potential damage, like changes in appearance or joint stiffness and deformities, especially in severe cases of morphea.

For patients with shallow, localized morphea, using topical treatments directly on the skin can be very effective. Topical corticosteroids are usually the first choice of treatment and are normally used for about 3 to 4 weeks. Topical tacrolimus 0.1% is another recommended treatment option for localized morphea.

In cases where morphea spreads widely and penetrates deeply, the prefered treatment is usually phototherapy using ultraviolet A1 (UVA1). UVA1 therapy penetrates more deeply than ultraviolet B (UVB) therapy and is less likely to cause sunburn. However, if UVA1 is not an option, other kinds of phototherapy like broadband UVA, narrowband UVB, and PUVA (a combination of psoralen and long-wave ultraviolet A) can be considered. It’s worth noting, though, that phototherapy may not be effective if the morphea has spread to deeper tissues, like the subcutaneous tissue (the layer of fat beneath the skin), muscles, or bones.

For patients who can’t access UVA therapy, high potency topical corticosteroids, corticosteroids injected into the skin, or topical tacrolimus may be used as treatments.

For lesions that are progressing rapidly, systemic therapy, which treats the whole body, may be required. This type of treatment often involves the use of corticosteroids or methotrexate, either orally or intravenously. These treatments have proven effective, but symptoms can relapse after stopping treatment. For such patients, combining methotrexate and systemic corticosteroids is often the first choice of treatment. Mycophenolate mofetil can be an alternative to methotrexate.

Surgeries like autologous fat grafting (using the patient’s own body fat to fill in areas), can be an option for patients with a certain type of morphea known as ‘en coup de sabre’.

It’s crucial to distinguish between morphea and systemic sclerosis. Systemic sclerosis affects the entire body and can lead to severe health problems, especially if it’s not detected early. People need to be checked for signs of this condition such as Raynaud’s phenomenon, skin tightening on fingers or toes, causes of hardened skin patches, and small, dilated blood vessels. If these signs are present, they need further screening through blood tests, skin microscopy and lung function tests.

Additionally, it’s important to separate morphea from other disorders that can cause skin to thicken and harden, such as scleromyxedema, lipodermatosclerosis, and radiation-induced morphea.

Scleromyxedema is often seen in patients suffering from systemic diseases like problems with plasma cells. A skin biopsy can reveal this condition by showing a spread of a substance called mucin, made primarily from an acid known as hyaluronic, in the upper and middle skin layers.
Radiation-induced morphea is another rare variation. This is a form of localized scleroderma that gradually worsens over time, and it occurs after exposure to radiation.

What to expect with Morphea

Morphea is a condition that causes patches on the skin. If these patches are only on the surface of the skin, they are usually not a big problem and the outlook is generally positive. However, some types of morphea can affect deeper layers of tissue, such as the layer of fat below the skin, muscle tissue, or even bone. This can lead to problems with movement and appearance.

Many children who have a certain type of morphea, called linear morphea, can develop issues like joint contractures, which is when the joints become stiff and hard to move, and limb length discrepancies, which is when one limb is shorter than the other.

Possible Complications When Diagnosed with Morphea

If left untreated, Morphea skin changes can lead to cosmetic issues, though it rarely affects internal organs. In children, a type of Morphea known as linear morphea might result in severe shrinkage of the arms or legs, joint stiffness and differences in limb length. Another form called ‘en coup de sabre’ can cause inflammation and hardening of deeper tissues, leading to uneven facial development and deformities.

Patients undergoing treatment need to be regularly checked for medication side effects, particularly if taking methotrexate. Routine lab tests are recommended every three months to ensure safety.

Common Issues:

Cosmetic alterations from skin changes
Severe shrinkage of arms or legs in children
Joint stiffness
Differences in limb length
Inflammation and hardening of deeper facial tissues
Uneven facial development and deformities
Side effects from methotrexate medication
Need for regular lab tests

Preventing Morphea

Morphea is a relatively uncommon skin condition that both kids and adults could potentially get. The cause of Morphea is thought to be related to an issue with the body’s immune system. However, it’s important for patients to be aware that factors like injuries, exposure to radiation, and even wear and tear from rubbing against something could also play a part. There seems to be a genetic factor as well, meaning that it could run in families, and research suggests that relatives of someone with Morphea might have a higher chance of having other immune system-related conditions.

Understanding one’s condition is a key part of managing it effectively, and this holds true for Morphea as well. By educating themselves about their condition, patients can better understand what to expect and make informed choices about their treatment options. If a doctor prescribes medications such as corticosteroids, methotrexate, or mycophenolate mofetil, it’s vital that patients also get information from a pharmacist. The pharmacist can explain typical side effects, how these medications might interact with other drugs, and the need for birth control methods to prevent pregnancy while on these medications. This can help increase patients’ willingness and ability to follow their prescribed treatment plan.

Frequently asked questions

Morphea, also known as localized scleroderma, is a rare skin condition that causes inflammation and hardening of the skin and the layer just beneath it. It can occur in both children and adults and comes in many different forms, ranging from small areas of skin affected with little discomfort to more serious forms that affect large areas.

The prevalence of Morphea ranges from 3.4 to 27 cases per 100,000 people. In the United States, there are 2 to 3 cases per 100,000 people.

The signs and symptoms of Morphea include: - Red skin patches in the early stages, which can be itchy and tender. - Hardening of the patches over time, with a purple border. - Changes in skin color and some shrinking of the skin and fatty layer underneath. - Some people may experience new patches and persistent symptoms even after a few years. - Different types of Morphea have specific symptoms: - Circumscribed Morphea: Separated round or oval spots, with a superficial or deep form. The deep form can cause limited joint movement. - Linear Morphea: Band-like patches on the limbs or around the neck and head, common in children. It can affect deeper tissues, leading to muscle weakness, joint limitations, and limb deformities. - En coup de sabre Morphea: Swooping patch on the forehead, extending to the scalp and causing hair loss. It can also cause eye and neurological symptoms if the deeper layers of skin are involved. - Generalized Morphea: Severe form with many patches or merged plaques, more common in women. It can affect joint movements and is defined by the presence of more than four patches in at least two different body parts. - There are also rare forms of Morphea like bullous and guttate Morphea. - Other similar conditions include eosinophilic fasciitis and Parry Robertson syndrome. - The term "mixed Morphea" is used when more than one subtype of Morphea is present simultaneously.

The cause of Morphea is still largely unknown, but it may be linked to genetic factors, issues with the immune system, and exposure to certain environmental factors.

The doctor needs to rule out the following conditions when diagnosing Morphea: - Systemic sclerosis - Scleromyxedema - Lipodermatosclerosis - Radiation-induced morphea

The types of tests that may be needed to diagnose Morphea include: 1. Physical checkup: A simple physical examination by a doctor can often lead to a diagnosis of Morphea. 2. Skin biopsy: In certain cases, a skin biopsy may be necessary. This involves taking a small sample of the skin, including the layer of fat beneath it, which will be analyzed to confirm the diagnosis. 3. Blood tests: Blood tests can be helpful in diagnosing Morphea. These may include tests for certain antibodies such as ANA, anti-histone Ab, and SS DNA Ab. Additionally, a blood test that shows an unusually high number of a certain type of white blood cell (eosinophilia) and markers indicating inflammation might aid in diagnosing the condition. 4. Magnetic Resonance Imaging (MRI): In severe cases of Morphea, an MRI can provide a clearer understanding of how deep the skin lesions go and if there are any complications, such as thickening of the connective tissue beneath the skin, inflammation of the tissue that lines the joints, or inflammation of the muscles. 5. Ultrasound: Ultrasound can also be used as an imaging method to monitor the disease, especially in routine monitoring. It's important to note that the specific tests needed may vary depending on the individual case and the severity of the condition.

Morphea can be treated in various ways depending on the specific kind of morphea, the depth of the condition, and the disease's activity. For shallow, localized morphea, topical treatments like corticosteroids or topical tacrolimus are often used. Phototherapy, specifically UVA1 therapy, is preferred for cases where morphea spreads widely and penetrates deeply. If UVA1 therapy is not an option, other types of phototherapy can be considered. High potency topical corticosteroids, corticosteroids injected into the skin, or topical tacrolimus may be used as treatments for patients who can't access UVA therapy. Systemic therapy, which treats the whole body, may be required for rapidly progressing lesions and can involve the use of corticosteroids or methotrexate. Surgeries like autologous fat grafting can be an option for a specific type of morphea called 'en coup de sabre'.

The side effects when treating Morphea can include cosmetic alterations from skin changes, severe shrinkage of arms or legs in children, joint stiffness, differences in limb length, inflammation and hardening of deeper facial tissues, uneven facial development and deformities, side effects from methotrexate medication, and the need for regular lab tests.

If the patches of Morphea are only on the surface of the skin, the prognosis is generally positive. However, some types of Morphea can affect deeper layers of tissue, leading to problems with movement and appearance. Children with linear Morphea can develop issues like joint contractures and limb length discrepancies.

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