Scleroderma is an autoimmune rheumatic disease that can cause changes in the skin, lungs, heart, and other body parts. Although scleroderma specifically affects collagen, other diseases also affect collagen and share similar symptoms to scleroderma.
Some of these diseases include systemic lupus erythematosus (SLE), polymyositis and dermatomyositis, systemic sclerosis, mixed connective tissue disease (MCTD), polymyalgia rheumatica (PMR) and Sjogren’s syndrome.
SLE, or lupus, is a chronic autoimmune disorder that affects the skin, joints, kidneys, heart and other organs. Symptoms of SLE include joint pain, fatigue, rash, fever and muscle pain.
Polymyositis and dermatomyositis are inflammatory muscle diseases that cause muscle weakness, skin rashes and joint pain. They are considered to be rare autoimmune disorders.
Systemic sclerosis is a connective tissue disorder that affects many organs, including the skin, lungs, kidneys and heart. Symptoms usually appear in the hands and feet first, and can include skin changes, joint pain and stiffness.
Mixed connective tissue disease is an overlap disorder of autoimmune diseases, commonly involving lupus, scleroderma, polymyositis and others. Symptoms of MCTD include joint pain, swelling and stiffness.
Polymyalgia rheumatica (PMR) is an inflammatory disorder that causes pain and stiffness in the shoulders and hips. Other symptoms include difficulty sleeping, fatigue and mild fever.
Sjogren’s syndrome is an autoimmune disorder that affects moisture-producing glands, leading to dry eyes and dry mouth. Other symptoms can include fatigue, joint pain and inflammation of multiple organs.
What can mimic scleroderma?
Mimics of scleroderma (also called systemic sclerosis) are diseases that may have similar clinical signs and symptoms to scleroderma but are actually caused by different underlying health conditions.
Including Raynaud’s phenomenon, eosinophilic fasciitis, Sjögren’s syndrome, lupus, and overlap syndrome.
Raynaud’s phenomenon is a condition that can be a sign of an underlying disorder, and it is characterized by episodic constriction of small arteries leading to the fingers, toes, ears, and nose. Symptoms include cold sensitivity, pain, numbness and tingling, and pale, blue, red, or white discolorations of the affected area.
Eosinophilic fasciitis is a rare condition characterized by inflammation and thickening of the skin caused by increased eosinophils invading the fascia, which is the tissue that lies between the skin and muscles.
Common symptoms include skin tightening, swelling, and redness of the affected area and joint pain.
Sjögren’s syndrome is an autoimmune disorder that causes several signs and symptoms, including dry eyes, dry mouth, fatigue, joint pain, and rashes. It is important to note that these symptoms may also occur in scleroderma and it is important to tell your doctor if these symptoms persist or worsen.
Lupus is an autoimmune disorder characterized by chronic inflammation of different parts of the body, especially in the joints, skin, kidneys, lungs, and brain. Common symptoms include fever, joint pain, rashes on the face, arms, and legs, hair loss, swollen glands, and fatigue.
Overlap syndrome is a term for a combination of two or more diseases such as scleroderma and lupus. People with overlap syndrome generally have the signs and symptoms of both underlying diseases. This makes it more challenging to diagnose because the symptoms may overlap and mimic scleroderma.
Overall, it is important to talk to your doctor if you are experiencing any signs and symptoms of scleroderma or any condition that may mimic it. Your doctor will be able to make an accurate diagnosis based on your medical history, physical examination, and lab tests.
What are the differentials of scleroderma?
Scleroderma is an autoimmune disorder that causes the skin and connective tissues to become thick and hard. The main differential diagnoses for scleroderma include systemic lupus erythematosus (SLE), systemic sclerosis overlaps, hypertrophic osteoarthropathy, eosinophilic fasciitis, Raynaud’s phenomenon, polymyositis, dermatomyositis, polymyalgia rheumatica, myositis ossificans, and Sjögren’s syndrome.
In SLE,autoantibodies cause inflammation in multiple organs and the skin. Systemic sclerosis overlaps occurs when a patient presents with features of both SLE and scleroderma. Hypertrophic osteoarthropathy is a rare disorder that affects the long bones in the body and may also be accompanied by pulmonary disease.
Eosinophilic fasciitis causes skin fibrosis and affects subcutaneous tissue. Raynaud’s phenomenon is a condition characterized by episodes of constricted blood flow to the fingers and toes. Polymyositis, dermatomyositis, and polymyalgia rheumatica are autoimmune disorders that cause muscle inflammation and weakness.
Myositis ossificans is a rare condition that leads to the formation of bone in muscle tissue. Finally, Sjögren’s syndromecan cause dry eyes and mouth in addition to systemic inflammation.
What is the difference between scleromyxedema and scleroderma?
Scleromyxedema and scleroderma are two conditions that may both present with thickened and/or fibrotic skin, but they are two distinct diseases caused by different underlying mechanisms.
Scleromyxedema is a rare skin condition characterized by localized deposition of mucin extravasation and proliferation of a form of lymphocyte called plasmacytes, which produce a combination of mucin and collagen.
It is largely unknown what the primary cause of scleromyxedema is, but it is assumed that it tends to be autoimmune in origin. It is characterized by diffuse, pink-gray, thickened and often verrucous plaques that tend to arise on the body, extremities and face.
Pruritus or burning sensation may also be present in advanced stages of scleromyxedema.
In contrast, scleroderma is a progressive autoimmune disorder with diffuse thickening of skin and thickening of related structures such as fascia, blood vessels, and internal organs. Scleroderma is caused by immune cells that produce an abundance of collagen, leading to thickening of the skin and underlying structures.
It is of two major types: localized scleroderma and systemic scleroderma. Depending on the type and the location of the affected skin, it can cause stiffness and disfigurement, and can often cause irreversible joint damage.
It affects both men and women and is more common in people of African-American descent. It is characterized by thickened, shiny skin that often has tightness and stiffness.
Thus, while scleromyxedema and scleroderma are both skin conditions characterized by thick skin, they are two distinct diseases with different underlying mechanisms.
What mimics systemic sclerosis?
Systemic sclerosis (also known as scleroderma) is an autoimmune condition that affects the skin and the organs inside the body. While it is not completely understood, it is believed that the body’s immune system mistakenly attacks healthy tissues in the skin and organs which can cause damage and scarring.
Although systemic sclerosis is a unique condition, there are a few other conditions that may mimic its symptoms.
One condition is mixed connective tissue disease (MCTD). MCTD is an overlap syndrome that includes features of lupus, scleroderma and polymyositis. It can cause Raynaud’s phenomenon and skin changes that look a lot like scleroderma but it can also cause other symptoms like a dry, irritated scalp, swollen fingers and muscle pain.
Other autoimmune diseases, such as lupus, can also present with similar symptoms. Lupus is a chronic autoimmune disease that can affect the skin, joints and organs as well. It can cause a distinctive skin rash, hair loss, joint pain, fatigue and fever.
Finally, there is calcinosis circumscripta. This rare condition can occur in people with scleroderma and it involves the buildup of calcium deposits in the skin. Calcinosis circumscripta can cause skin cracking, itching, pain, and ulcerations.
Overall, while systemic sclerosis is a unique condition, several other conditions may share similar symptoms which can make diagnosis a bit challenging. If you are showing any signs of systemic sclerosis or are experiencing any of the symptoms listed above, it is important to contact your physician right away.
How do I know if I have systemic scleroderma?
Systemic scleroderma is a chronic condition that affects connective tissue, most often characterized by hardening and tightening of the skin and sometimes of the organs and blood vessels. To diagnose scleroderma, you should see your physician who will take a detailed medical history, review symptoms and perform a physical examination to determine if scleroderma is present.
Your doctor may order laboratory tests and specific imaging studies to evaluate for scleroderma. Common findings include thickening of the skin, abnormalities of the hands and nails, and the emergence of certain auto-immune markers in the blood.
They may also perform specific scans and tests such as nailfold capillaroscopy, antinuclear antibody tests, and echocardiography, to check for signs of scleroderma and possible damage to the heart. In some cases, more advanced tests such as bronchoalveolar lavage, esophageal manometry, and a skin biopsy may be performed to confirm the presence of scleroderma.
Along with a physical exam and diagnostic tests, your doctor can discuss your options for care and treatment.
What is differential diagnosis of morphea?
Differential diagnosis of morphea includes systemic sclerosis, lupus erythematosus, lichen sclerosus, atrophoderma, necrobiosis lipoidica diabeticorum, post radiation fibrosis, systemic lupus, collagen vascular diseases, degenerative disorders and skin diseases.
In addition, morphea may also be the result of response to a specific trigger, such as an infection or physical trauma. A full history and physical exam are essential to differentiate the diagnosis.
It is also important to consider laboratory studies, such as a complete blood count, urinalysis, chest x-ray or skin biopsy, depending on symptoms, as morphological criteria can provide further clues in the diagnosis of morphea.
Therefore, differential diagnosis of morphea requires a comprehensive evaluation, including clinical findings, laboratory results and imaging studies.
Is CREST the same as scleroderma?
No, CREST is not the same as scleroderma. CREST is an acronym for the five major symptoms associated with limited scleroderma, which is a rare form of scleroderma. The acronym stands for Calcinosis, Raynaud’s Phenomenon, Esophageal Dysmotility, Sclerodactyly, and Telangiectasia.
Generally, scleroderma refers to a disease of the skin and connective tissue, causing thickening and hardening of the skin. Limited scleroderma is associated with less serious symptoms than other kinds of scleroderma and only affects the skin.
It is generally localized to the hands, arms, and face. Symptoms of limited scleroderma can include tight or thickened skin, changes in the nails, hair loss, Raynaud’s phenomenon, telangiectasia, sclerodactyly (the tightening of the skin on the fingers), and calcinosis (the deposit of calcium in the skin).
In some cases, limited scleroderma can cause gastrointestinal and pulmonary problems. It is important to distinguish between CREST and scleroderma so that if you are experiencing any of the CREST symptoms, you can receive proper treatment.
How serious is CREST syndrome?
CREST syndrome (also referred to as limited scleroderma) is a rare, painful, and potentially disabling autoimmune disorder that can affect people of any age. It can affect the skin, blood vessels, and organs, causing a number of symptoms that can significantly interfere with quality of life.
In some cases, it can even be life threatening.
The severity of CREST syndrome varies from person to person. For some, it can remain mild with few or even no symptoms, whilst for others it can be more serious. In the most serious cases, it can cause organ damage and even death.
The exact cause of CREST syndrome is unknown, although it is thought to be related to an abnormality in the body’s immune system. Treatment options depend on the type and severity of the symptoms, but can range from medications, physical therapy, and lifestyle changes, to more intensive treatments such as steroids or immunosuppressants.
It is important to note that there is no cure for CREST syndrome, and the only way to manage it is to closely monitor and treat the symptoms as they arise.
In summary, CREST syndrome is a serious condition that can cause major disruptions to quality of life. For some, it can be mild and can be effectively managed with lifestyle changes and medications. But for others, it can be life-threatening, and close monitoring and intensive treatments may be necessary.
Is CREST syndrome an autoimmune disease?
No, CREST syndrome is not an autoimmune disease. Rather, it is a systemic sclerosis disorder, sometimes referred to as limited scleroderma. This disorder is characterized by thickening and hardening of the skin and other connective tissues, typically in the arms and legs, along with Raynaud’s phenomenon (where blood vessels in the extremities constrict when exposed to cold temperatures), calcinosis (accumulation of calcium in the skin and/or deeper tissues), telangiectasias (visibly enlarged blood vessels on the face and hands), esophageal dysmotility (difficulty swallowing), and pulmonary hypertension (high blood pressure in the lungs).
While the cause of CREST syndrome is unknown, it is likely caused by a combination of genetic and environmental factors. Treatment usually consists of controlling symptoms and preventing further progression of the disease.
How long is life expectancy with CREST scleroderma?
The answer to this question is not an exact one as it can vary greatly among individuals. Generally, life expectancy with CREST (or limited cutaneous systemic scleroderma) is typically higher than with diffuse systemic scleroderma.
Most studies have found that life expectancy for CREST patients is about 7-10 years after diagnosis, with some studies suggesting even higher life expectancies of up to 20 years.
However, as with all forms of scleroderma, life expectancy is highly dependent on many factors, including the severity of symptoms and the overall health of the patient. Other factors such as age and overall health can also influence life expectancy, as individuals with a weaker immune system or chronic illnesses may not fare as well as a healthier individual who has been diagnosed with CREST.
Overall, life expectancy with CREST scleroderma is ultimately determined by the specific circumstances of the individual. If a patient is able to successfully manage their symptoms and even attain remission in some cases, they may have a longer life expectancy than those who are not able to do so.
As such, it is important for individuals suffering from CREST scleroderma to take proactive steps towards managing their symptoms and leading a healthy lifestyle to increase their chances of a longer life.
What is the life expectancy of someone with CREST?
The life expectancy of someone with CREST (also known as CREST syndrome or limited scleroderma) varies based on the severity of the condition and the individual’s overall health. Generally, however, the prognosis for patients with CREST is good.
Most people with CREST have a normal life expectancy.
The most serious complications of CREST are due to the inflammation of the small blood vessels in the skin and internal organs, which can cause Raynaud’s phenomenon and pulmonary arterial hypertension (PAH).
Raynaud’s phenomenon can cause pain and damage to the fingers, toes, and other body parts as a result of reduced blood flow. PAH can lead to organ damage and, in extreme cases, death.
However, medical advancements such as improved non-steroidal anti-inflammatory medications, biologic agents, and calcium channel blockers all greatly reduce the risk of developing severe or fatal complications.
As a result, the prognosis of someone living with CREST is often very good, and with proper care and management, the life expectancy should be roughly the same as someone without CREST.
What are the stages of CREST syndrome?
CREST syndrome is a rare disease that affects around 100,000 people worldwide, and is characterized by a combination of five symptoms known as the CREST syndrome (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia).
Due to the variety of these symptoms and the fact that they may all appear at once or separately over time, determining the stage of CREST syndrome can be difficult.
The stages of CREST syndrome can generally be broken down into two distinct phases; the progression phase and the resolution phase.
The progression phase of CREST syndrome typically involves the development of the five main symptoms of the condition, gradually increasing in severity over time. During this stage, an influx of hardening skin deposits, known as calcinosis, will usually begin to form under the skin.
Raynaud’s phenomenon is also common and may cause episodes of coldness, tingling, and color changes on the fingers and toes when exposed to cold temperatures. Other signs can include difficulty swallowing as a result of esophageal dysmotility, thickening or hardening of the skin on the fingers or toes (sclerodactyly), and visible, reddish-purple patches or small vessels on the skin due to telangiectasia.
The resolution phase of the disorder is characteristically marked by the disappearance of the five classic CREST syndrome symptoms. With treatment and vigilant management of the disorder, the progression of the condition may cease, leaving the affected area with smooth and soft skin, as well as normal temperature sensation in the fingers and toes.
Calcinosis, however, may still remain in areas that have previously been affected by CREST syndrome.
Overall, CREST syndrome is a complex and rare disorder that can be difficult to diagnose and manage. It is important to speak with a doctor or specialist in order to confirm the diagnosis and begin treatment as soon as possible.
Does CREST syndrome always progress?
No, CREST Syndrome does not always progress. CREST Syndrome is the acronym for Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia, which are the five clinical features considered collectively.
While CREST Syndrome can gradually cause tissue in such areas as hands, feet, and face to become progressively hardened, the time frame and degree to which it progresses varies from person to person.
Factors such as the type of treatment received and lifestyle habits can also have an effect on whether CREST Syndrome progresses and how quickly. Therefore, while it is possible for CREST Syndrome to progress, it is not a certainty.
Can lupus be mistaken for scleroderma?
Yes, lupus and scleroderma can be mistaken for one another due to their similarities. Both conditions are autoimmune diseases, meaning they are characterized by the body’s immune system attacking healthy cells as if they are foreign or diseased.
Symptoms often associated with both disorders can include fatigue, joint pain, and skin rashes, which can lead to confusion in diagnosing the right condition. However, there are some subtle differences that can help differentiate between lupus and scleroderma.
While lupus is often characterized by rashes that look like butterfly-shaped lesions across the cheeks and nose, scleroderma will often cause thickened skin on the hands, arms, legs, and face. Furthermore, scleroderma is associated with inflammation and hardening of the internal organs, such as the esophagus, lungs and kidneys, whereas lupus does not typically cause these.
Other symptoms of lupus can include hair loss, anemia, and fever, and scleroderma often shows itself as Raynaud’s Syndrome, a disease caused by poor circulation in the extremities, and distorted nail beds.
Ultimately, lupus and scleroderma do have their similarities, but there are also some distinct characteristics that may help physicians accurately diagnose and treat these conditions.