The three types of scleroderma are localized (limited), diffuse, and systemic sclerosis. Localized scleroderma, sometimes referred to as limited scleroderma, is the mildest type of scleroderma and usually affects the skin of the face, hands, and feet.
It does not usually affect the internal organs and rarely progresses. Diffuse scleroderma affects the skin and internal organs, including the gastrointestinal tract, lungs, and kidneys, and can cause progressive joint contractions, or tightening.
It is the most serious type of scleroderma and can be life-threatening. Systemic sclerosis also affects the skin, internal organs, and joints, but it progresses more slowly than diffuse scleroderma and is considered a more severe but less life-threatening form.
It can be more difficult to diagnose due to its slower rate of progression.
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How do you know what type of scleroderma you have?
In order to determine which type of scleroderma you have, you will need to be assessed by a medical professional. Your doctor or a rheumatologist will be able to diagnose you and determine which type of scleroderma you have after a comprehensive evaluation.
They will review your medical history, family history, and conduct a physical exam to assess any visible signs of the condition. Additionally, blood tests may be conducted to test for levels of antibodies that are associated with certain types of scleroderma, x-rays can be taken to examine the lungs, and other imaging technologies may be used assess the internal organs and structures.
Once all of the exams, tests, and scans have been evaluated, your doctor will be able to make a formal diagnosis of your type of scleroderma.
How do you know if you have localized or systemic scleroderma?
Localized scleroderma, also known as morphea, is a type of autoimmune disorder that affects the skin. It is caused by an abnormal reaction of the body’s immune system and results in patches of hardened, thickened skin.
This can sometimes affect the underlying tissue, muscle, and even bone.
Signs and symptoms of localized scleroderma may include patches of hardened, thickened skin, often on areas of the trunk and arms; patchy hair loss where affected skin patches appear; and sometimes, discolouration of the affected skin.
In some cases, localized scleroderma can also cause joint stiffness, muscle weakness, and limited range of motion.
Systemic scleroderma, also known as systemic sclerosis, is a type of autoimmune disorder, similar to localized scleroderma, that affects multiple organs and body systems including the skin, heart, lungs, and gastrointestinal tract.
As systemic scleroderma progresses, scar-like tissue forms in many parts of the body, eventually leading to hardening of the skin, tightening of the blood vessels, thickening of the lungs, and fibrosis of the heart, abdomen, and muscles.
Signs and symptoms of systemic scleroderma may include patches of hardened, thickened skin in areas of the face, neck, chest and hands; aching, swollen or tender finger joints; issues with breathing; a pale or blue coloration of the fingertips (due to diminished vascular circulation); painful and/or swollen areas around the elbows and knees; digital ulcers on the fingers and toes; and gastro-esophageal problems (reflux and swelling of the esophagus).
In order to accurately diagnose localized or systemic scleroderma, a trained medical professional will conduct a physical examination and review your medical history. They may also request lab tests such as blood tests, CT scans, x-rays, and MRI scans.
In some cases, a skin biopsy may also be recommended to further assess the extent of the condition.
What is the difference between diffuse and limited scleroderma?
The main difference between diffuse and limited scleroderma is the area of the body that is affected. Diffuse scleroderma is a systemic disorder that affects the whole body, including the skin, joints, muscles, and organs.
Symptoms may include skin tightening, digestive issues, fatigue, and joint stiffness or pain. Limited scleroderma, or CREST syndrome, is named for its most common symptoms which are Calcinosis (calcium deposits in the skin or joints), Raynaud’s phenomenon (a condition in which the fingers and toes experience episodes of decreased circulation and extreme cold), Esophageal Dysfunction (difficulty swallowing), Sclerodactyly (thickening and hardening of the skin on the fingers and toes), and Telangiectasia (red spots on the skin caused by enlarged blood vessels).
While both sclerodermas cause similar skin tightening, limited scleroderma is limited to the skin and joints, and does not affect the organs in the same way diffuse does.
What ANA pattern is associated with scleroderma?
The American-European Consensus Group (AECG) has classified seven distinct antinuclear antibody (ANA) patterns in association with scleroderma. These patterns are known as centromere pattern, homogeneous pattern, nucleolar pattern, cytoplasmic pattern, speckled pattern, centromere/nucleolar pattern, and systemic sclerosis-specific pattern.
The centromere pattern is characterized by a diffuse nuclear positivity composed of fine speckles and bigger dots scattered around the rim of the nucleus. This is arguably the most common ANA pattern associated with scleroderma, particularly in localized forms of the disease.
The homogeneous pattern consists of a diffuse nuclear positivity, in which the fine speckles and dots are evenly distributed throughout the nucleus. This pattern is usually seen in the early phases of scleroderma, particularly in limited forms of the disease.
The nucleolar pattern includes a strong positivity of the nucleoli, without any associated nuclear staining. This pattern is typically seen in more diffuse forms of scleroderma and is particularly associated with the presence of a cutaneous and/or systemic-serologic overlap syndrome.
The cytoplasmic pattern is characterized by a strong positivity in the cytoplasm without any associated nuclear staining. This pattern is often associated with diffuse forms of scleroderma and is also seen in overlapping syndromes.
The speckled pattern is characterized by a fine speckled nuclear positivity, in which the dots are arranged in multiple groups. This pattern is seen in the early stages of scleroderma and is sometimes seen in overlapping syndromes, such as scleroderma renal crisis.
The centromere/nucleolar pattern consists of a strong staining of both the centromere and the nucleoli, without any associated nuclear positivity. This pattern is usually seen in the late stages of scleroderma and is most commonly associated with the presence of severe disease.
The systemic sclerosis-specific pattern is characterized by a diffuse nuclear positivity composed of fine speckles and larger dots scattered around the rim of the nucleus. This pattern is seen only in patients with systemic sclerosis and is associated with the presence of an anti-topoisomerase I antibody.
Which autoantibody is most specific for scleroderma?
The autoantibody most specific for scleroderma is the anticentromere antibody (AECA). This antibody is thought to be more specific for scleroderma than other autoantibodies, as it is found in approximately 95% of people with limited cutaneous systemic sclerosis and around 75% of people with diffuse cutaneous systemic sclerosis, and is virtually absent in people without the condition.
Additionally, while other autoantibodies may also be present in people with scleroderma, the presence of antiproteinase 3 antibodies and antinuclear antibodies (ANA), while important as part of a differential diagnosis, are not as specific indicators for scleroderma as AECA.
How does a rheumatologist diagnose scleroderma?
A rheumatologist may use a variety of tests to diagnose scleroderma. These may include a physical exam and laboratory tests, such as blood tests, X-rays and ultrasounds. During the physical exam, the rheumatologist may look for typical scleroderma symptoms like hard or thick patches of skin, stiffness of the joints, esophageal reflux, and vascular symptoms.
During the laboratory tests, the rheumatologist may test for antibodies such as antinuclear antibodies, anti-RNA polymerase III and anticentromere to help diagnose scleroderma. In addition, a skin biopsy may be taken to examine the skin under a microscope and make a definitive diagnosis.
Imaging tests may be performed to look at the organs and the small blood vessels in the skin, in order to identify areas that may be affected by scleroderma. The rheumatologist may also use pulmonary function tests to evaluate the lungs and an electrocardiogram to monitor the heart’s electrical activity.
Depending on the results of all of these tests, the rheumatologist can then diagnose and treat scleroderma.
What is the scleroderma skin pinch test?
The scleroderma skin pinch test is a type of test used to evaluate skin thickness. It is a simple exam wherein the assessor takes hold of and pinches a piece of skin between their thumb and index finger and then measures how long it takes until the skin bounces back to its original state.
This test is especially useful for those with scleroderma, an autoimmune condition that thickens the skin and can cause other symptoms such as joint pain, stiffness, and fatigue. It is also a great clinical tool to help indicate to physicians if a person is in the early or late stages of the condition.
A quicker recovery time would mean an early-stage scleroderma, a slower or delayed recovery time would likely imply a more advanced form of the condition. In addition to offering insight into the condition’s stage, the pinch test also helps physicians differentiate between scleroderma and other skin diseases with similar symptoms, such as Systemic Lupus Erythematosus (SLE).
Where does scleroderma usually start?
Scleroderma usually starts in the skin and can occur in any area of the skin, such as the hands, face, or feet. It can spread to other body parts, such as the lungs, heart, gastrointestinal system, or kidneys.
It can affect the joints and muscles, leading to joint and muscle stiffness, pain, and fatigue. Scleroderma can also cause Raynaud’s phenomenon, or the narrowing and widening of blood vessels due to extreme cold or stress, which can cause numbness or pain in the fingers, toes, or other extremities.
People living with scleroderma often complain of dry eyes, dry mouth, and digestive problems.
Does scleroderma come on suddenly?
No, scleroderma does not come on suddenly. It typically develops gradually over time. Usually, the signs and symptoms of scleroderma can appear slowly and progress for months or even years before the exact diagnosis is made.
In some cases, scleroderma can begin abruptly and progress quickly, but this is relatively rare.
Typical signs and symptoms of scleroderma may include localized thickened skin, tightness and stiffness in the joints, Raynaud’s phenomenon (changes in skin color in response to cold temperatures or stress), fatigue, and lifestyle issues such as sleeping problems, digestive issues and depression.
With progressive scleroderma, the skin and blood vessels can become hardened and fibrotic, leading to organ damage if it is not treated.
If you are experiencing any of the above symptoms, be sure to consult with your doctor as soon as possible. They will be able to properly diagnose the condition and put you on the right path to treatment and management.
How fast does scleroderma progress?
Scleroderma is an autoimmune disorder that affects connective tissues throughout the body. It is progressive, meaning that its symptoms and impact can worsen over time. The speed at which scleroderma progresses varies, and can depend on different factors.
It is important to note that the progression of scleroderma can be different for each individual.
In general, the disease can progress quickly over the course of a few months to a couple of years. However, it can also progress slowly over the course of several years. Factors such as a person’s age, overall health, and lifestyle can also affect how quickly scleroderma progresses.
If you are living with scleroderma, it is important to talk to your healthcare provider about your individual risk factors and how quickly the disease may progress for you. It is also important to receive regular checkups and follow-up care to keep track of how the disease is progressing and take steps to manage or slow its progression.
At what age is scleroderma usually diagnosed?
Scleroderma is a medical condition that can affect people of all ages. However, it is usually diagnosed between the ages of 30 and 50. The disease affects women more than men, with women accounting for 80-90% of all cases.
While the exact cause of scleroderma is unknown, it is believed that genetics, environmental exposures, and the body’s response to an underlying infection may play a role.
Those with scleroderma may experience an abnormal hardening of the skin, and the hardening may affect other parts of the body such as the blood vessels, joints, and internal organs. Symptoms can vary depending on the type of scleroderma and can include joint pain and stiffness, Raynaud’s phenomenon (in which the fingers or toes turn white or blue in response to cold temperatures), swollen fingers, impaired mobility in the hands or face, and ulcers on the fingers or toes.
Some patients may also experience trouble breathing, or a swelling of the stomach due to an enlarged esophagus.
Due to the heterogeneous nature of scleroderma, diagnosis can sometimes be difficult and requires evaluation by a physician knowledgeable in autoimmune and connective tissue disorders. The physician may perform physical exams, lab tests, blood tests, skin biopsies, lung function tests, and MRI or CT scans to diagnose scleroderma.
In conclusion, scleroderma can affect individuals of all ages, but most commonly appears between the ages of 30 and 50. Diagnosis of the condition depends on the patient’s individual symptoms and can be made by a skilled physician after evaluation.
What can be misdiagnosed as scleroderma?
Scleroderma can be confused with other conditions because some of its symptoms are similar to those of other autoimmune diseases. Examples of conditions that can be misdiagnosed as scleroderma include Raynaud’s phenomenon, Sjogren’s syndrome, dermatomyositis, lupus, rheumatoid arthritis, and polymyositis.
Other conditions that may be misdiagnosed as scleroderma include Lupus erythematosus, myasthenia gravis, and diphtheria. These conditions share symptoms with scleroderma that can lead to misdiagnosis.
Raynaud’s phenomenon is a common condition that is often misdiagnosed with scleroderma because of its similar symptoms. Raynaud’s causes the vessels in the body to overreact to cold temperatures and other stressors, resulting in episodes of spasms and changes in the skin color.
Sjogren’s syndrome is an autoimmune disorder that can also be mistaken for scleroderma because of similarities in symptoms. Those with Sjogren’s syndrome experience dryness in the mouth and eyes, which can resemble scleroderma’s symptoms of dry, tight skin.
Dermatomyositis is another condition that can be mistaken for scleroderma due to overlapping symptoms. Those with dermatomyositis experience weakness in the muscles, skin rash, and swollen hands, which can be misdiagnosed as scleroderma.
Lupus and rheumatoid arthritis can also be misdiagnosed as scleroderma. With both of these conditions, skin is more sensitive to sunlight, and one may experience joint stiffness, which can lead to misdiagnosis.
Lastly, polymyositis can also be misdiagnosed as scleroderma. Those with polymyositis experience muscle pain and tenderness, which can be misdiagnosed as scleroderma’s skin tightness and stiffness.
It is important to remember that scleroderma is unique and complicated and many conditions have overlapping and sometimes confusing symptoms. If you think that you may be experiencing symptoms of scleroderma, it is important to speak to a doctor and get tested to be sure that you receive proper diagnosis and treatment.
Can scleroderma be detected in blood test results?
Yes, it is possible to detect scleroderma in blood test results. Some of the tests that may be used to diagnose or monitor individuals with scleroderma are C-reactive protein (CRP), ANA or antinuclear antibody test, sedimentation rate, and complete blood count (CBC).
In addition, the ESR, prothrombin time (PT) and activated partial thromboplastin time (aPTT) may also be done to evaluate how the body’s ability to form blood clots is affected. In some cases, a tissue biopsy may be done to confirm the diagnosis.
Additionally, ultrasounds can be used to look for changes in the blood vessels, which can sometimes be seen in scleroderma. In more advanced stages of the condition, patient may require an angiogram or MRI scans to check for thickening of the walls of major blood vessels.
Ultimately, the best way to determine if a person has scleroderma is to involve a qualified doctor and undergo the appropriate tests.
Is scleroderma hard to diagnose?
Yes, scleroderma can be difficult to diagnose in some cases. Symptoms can vary from one individual to the next, and the severity of symptoms can be unpredictable. It is also possible for symptoms to come and go, making it challenging for doctors to accurately identify and diagnose the condition.
Additionally, not all testing for scleroderma is reliable, which can make diagnosis difficult. To properly diagnose scleroderma, a doctor might take a biopsy of the skin and perform blood tests to look for antibodies associated with the condition.
The doctor will also likely review the patient’s medical history and lifestyle, looking for any signs that point to a diagnosis of scleroderma.
