What is Scleredema?
Scleredema of Buschke is a rare disease that affects the body’s connective tissues, which are responsible for holding our body parts together. The exact cause of this condition is not fully understood. The disease was first noted by Curizo in 1752 and later defined by Buschke in 1902. It is classified under diseases similar to scleroderma, a condition where the skin becomes hard and tight.
People with Scleredema of Buschke experience a slow, progressive hardening of the skin, usually affecting the neck, shoulders, upper limbs and occasionally the face. This condition often appears following an infection. It has also been found in connection with diabetes and, in rare cases, blood disorders.
When looking at the disease under a microscope, the skin shows fibrosis, a process where the skin thickens due to excessive growth of fibrous connective tissue. The skin also has thickened collagen bundles, proteins that provide structure to the skin, and varying amounts of a jelly-like substance called mucin.
What Causes Scleredema?
Buschke’s scleredema is categorized into three types since 1968, according to Graff’s classification.
Type 1 scleredema is the most common, accounting for about 55% of cases. It mostly affects children and young adults. Often, after having a fever or infectious illness like a streptococcal throat infection or a less common viral illness, the scleredema appears suddenly about 2 to 3 weeks later.
Type 2 scleredema makes up about 25% of cases. It starts subtly and progresses slowly, often associated with unusual blood conditions. Paraproteinaemia, a condition with too much protein in the blood, is the most common and usually develops many years after the scleredema starts. It happens at a younger age compared to the general population. Other linked conditions include overly high levels of IgG and IgA (types of immune proteins), multiple myeloma (a cancer of plasma cells), and amyloidosis (a buildup of abnormal proteins in the organs). However, the connection between scleredema and extra protein in the blood is still unclear.
Type 3 scleredema, also known as scleredema diabeticorum, affects people with diabetes and it makes up around 20% of cases. It’s a common complication among those who have had poorly managed insulin-dependent diabetes for a long time. Severe diabetic blood vessel complications are usually associated with this condition. It affects 2.5% to 14% of all diabetic patients. There’s a theory suggesting that high sugar levels can cause nonenzymatic glycosylation of collagen in the skin, which means sugar attaches to collagen protein causing changes in the skin structure.
Other conditions listed alongside Buschke’s scleredema include primary hyperparathyroidism, rheumatoid arthritis, ankylosing spondylitis, Sjogren syndrome, dermatomyositis, Waldenstrom macroglobulinemia, anaphylactoid purpura, primary biliary cirrhosis, and IgA deficiency. There have also been cases reported of lichen sclerosus and atrophicus and scleredema adultorum of Buschke occurring at the same time, suggesting that conditions that make skin hard and tight might share a common cause. Rare cases of Buschke’s scleredema have been reported along with cancers.
Risk Factors and Frequency for Scleredema
Scleredema of Buschke is a disease whose frequency among the general population is not clearly known. However, it’s important to understand that this disease could affect anyone, regardless of their age or race. That being said, we can see some patterns when we look closer at various types of this disease.
- The first type mainly affects children and young adults.
- The second and third types are more common among elderly patients, specifically those over forty years old.
- Men are more likely to get types 1 and 2, with a sex-ratio of about 2 to 1.
- However, for type 3, the ratio is significantly higher, with about 10 men affected for every woman.
Signs and Symptoms of Scleredema
Buschke Type 1 Scleredema is a skin condition that usually comes on suddenly, often following an illness with a fever in the past two to three weeks. This should point towards the correct diagnosis. On the other hand, Type 2 and 3 Scleredema may take longer to diagnose because their symptoms develop slowly over several years. You might see a circular rash or skin redness before the skin becomes hard. It can also cause itchiness, and less frequently, pain. People might typically complain about difficulties in moving their shoulders and jaw, and in chewing.
During a physical examination, the skin will often seem hard, like wood, and it won’t pit (keep a dent) when pressed. There might be some redness or darkening of the skin, but this isn’t always the case. The disease first shows up on the neck before spreading symmetrically to the shoulders, upper torso, and arms. In some cases, it may also affect the face, buttocks, and thighs, but it doesn’t usually involve the hands or feet. The affected skin may take on a rough, dimpled appearance similar to an orange peel when pinched. There have also been reports of severe cases where it affects the entire thick layer of skin. Unusual cases might present with sudden, painless swelling around both eyes.
It’s rare for Scleredema to impact anything other than the skin, but especially in Types 2 and 3, it might affect organs and parts of the body like the tongue, heart, lungs, muscles, esophagus, salivary glands in the mouth, liver, spleen, pleural membranes lining the lungs, and eyes. Complications of this can include restricted movement, difficulty swallowing, dryness in the mouth and eyes, and fluid in the organs or body cavities. Alongside this, careful attention should be paid to any signs of cancer or severe diabetes complications.
Testing for Scleredema
The process of diagnosing scleredema, a skin condition where the skin thickens, is based on examining the symptoms and the physical characteristics of the skin. There’s no need for lab tests or immune system checks. However, doctors usually recommend a screening for a protein called paraprotein. This is because its presence can suggest a disorder where your body produces too many lymphocytes, a type of white blood cell.
Additionally, the thickness of the skin can be assessed with an ultrasound. The ultrasound is important as it helps doctors determine the extent and seriousness of scleredema.
Treatment Options for Scleredema
Scleredema of Buschke is a condition for which there isn’t a specific treatment. However, we have several ways to relieve its symptoms. The first line of treatment often involves the use of light therapy, otherwise termed as phototherapy.
If light therapy doesn’t work or if the condition is severe, doctors may suggest intravenous immunoglobulin. This is a product made up of antibodies that can boost your body’s immune system response.
It’s also important to treat the root cause of this condition. With type 1 scleredema, which can often be triggered by a streptococcal infection, antibiotics will be needed. If you have type 2, you’ll need care from a hematologist, a doctor who’s an expert in blood conditions. Tight management of diabetes is advised for those with type 3, however, it’s worth noting that this doesn’t always improve the scleredema.
Other potential treatments include steroids, cyclosporine (a drug used to suppress the immune system), penicillamine (a medication mostly used for arthritis), cyclophosphamide (a type of chemotherapy), electron beam therapy, high doses of intravenous penicillin and others.
Unfortunately, none of these approaches consistently solve the problem and many patients only experience partial relief. If the scleredema is causing limited mobility, physical therapy may be recommended to improve movement and function.
What else can Scleredema be?
When diagnosing scleredema of Buschke, a skin condition leading to hardening and thickening of the skin, other diseases that also cause swelling and mucin build-up should be considered. These often include conditions like scleromyxedema and scleroderma.
Scleroderma is often seen in a disease known as systemic sclerosis. This condition usually causes an irregular thickening of the skin and changes in color. It often progresses from the center of the body outward. Other symptoms can include specific autoantibodies (proteins that attack your own body), changes in how your nails look, and abnormal blood flow in the tiny capillaries of your fingers. The primary feature of scleroderma is the excessive collagen leading to thick skin. However, the main distinction between scleredema and scleroderma often comes from the symptoms rather than findings seen under a microscope.
Scleromyxedema, is another similar disorder to scleroderma. Both conditions cause an increase in mucin, a substance that helps keep your skin moist, but this can lead to hardening and thickening of the skin. Scleromyxedema, in particular, causes small, hard skin bumps that can grow and form hardened areas, particularly on the face, neck and arms. Unlike scleredema, it can also affect the hands and fingers. Under the microscope, scleromyxedema is known by fibrosis (thickening and scarring of connective tissues) with separated dermal fibers due to excessive mucin deposits. Systemic (affecting the whole body) involvement is also common in scleromyxedema, due to the mucin deposits and it may also be associated with conditions like monoclonal gammopathy (protein produced in large amounts by an abnormal type of antibody), multiple myeloma (cancer of plasma cells), and thyroid disorders.
What to expect with Scleredema
The chances of getting better from Buschke’s scleredema, a skin condition, greatly hinge on the subtype of the disease. In individuals with type 1, the condition usually goes away on its own within 6 to 24 months. Although, there have been some cases where it took up to ten years. Unfortunately, for people with type 2 and 3, the disease tends to progress slowly. Over time, it may even start affecting other parts of the body and lead to life-threatening complications.
