What is scleromyxedema?
Scleromyxedema, also known as diffuse/generalized and sclerodermoid lichen myxedematosus or Arndt-Gottron disease, is a primary cutaneous mucinosis characterized by a generalized, papular and sclerodermoid, cutaneous eruption that usually occurs in association with monoclonal gammopathy [1].
Is scleromyxedema fatal?
Scleromyxedema is described as an unpredictable disease, but is usually progressive, debilitating, and lethal in the absence of a successful treatment. Death may result from complications with extracutaneous involvement or treatment side effects.
Who treats scleromyxedema?
“Dermatologists are often the first specialists to see these patients and to make the diagnosis of this disabling and potentially fatal condition, whose therapy is challenging,” he said. From his European multi-center study of 30 patients with scleromyxedema and from a personal trial on eight patients, Dr.
What is the difference between scleroderma and scleromyxedema?
Scleromyxedema presents with 2-4 mm waxy papules distributed symmetrically on the arms, hands, and face. [2] It is differentiated from scleroderma by the papular component and the absence of teleangiectasia.
Is scleromyxedema an autoimmune disease?
Scleromyxedema (SM) is a rare disorder which initially presents with waxy skin stiffness and maculopapular lesions. It also has non-dermatologic manifestations, such as serum paraproteinemia and myopathies, and is sometimes associated with other autoimmune disorders.
Is Scleroderma an autoimmune?
Scleroderma is an autoimmune connective tissue and rheumatic disease that causes inflammation in the skin and other areas of the body.
Is Scleromyxedema an autoimmune disease?
How do you pronounce Scleromyxedema?
Pronunciation of the word(s) “Scleromyxedema”. – YouTube
What is best hospital to treat scleroderma?
The Johns Hopkins Scleroderma Center is one of the largest and most active in the world, seeing over 200 new patients each year.
What can be mistaken for scleroderma?
Diagnosis and Tests
Because it can affect other parts of the body — such as the joints — scleroderma may be initially mistaken for rheumatoid arthritis or lupus. After discussing your personal family medical history, your doctor will perform a thorough physical examination.
What is End Stage scleroderma?
This type of scleroderma is typically accompanied by shortness of breath, a persistent cough, and the inability to perform routine physical activities. End-stage scleroderma often causes pulmonary fibrosis and/or pulmonary hypertension, both of which can be life-threatening.
What blood test shows scleroderma?
Antinuclear antibody (ANA) testing is the most important blood test to screen for scleroderma and other connective tissue diseases.
What are the final stages of scleroderma?
What is the newest treatment for scleroderma?
Two drugs are currently FDA approved to treat lung scarring, or pulmonary fibrosis, that occurs as a result of scleroderma: nintedanib and tocilizumab.
What diet is best for scleroderma?
General Diet Recommendations:
Choose fresh, whole, minimally processed foods, without preservatives, artificial ingredients, or hydrogenated oils. If there are “chemical” sounding names in the ingredient list, avoid it. In general, the shorter the ingredient list, the better.
How long do scleroderma patients live?
Results: A total of 413 deceased patients was identified. Females were overrepresented 315 to 98; 265 had limited scleroderma, 90 diffuse and 22 overlap disease. Over 30 years, the mean age of death improved from 66.4 to 74.5 years (P < 0.001). Duration of disease improved from 12.1 to 22.9 years (P < 0.001).
Can you live a long life with scleroderma?
In general, patients with limited scleroderma have a normal life expectancy. Some have problems with their GI tract, especially heartburn; severe Raynaud’s and musculoskeletal pain; and a small subset can develop pulmonary hypertension that can be life-threatening.
What virus causes scleroderma?
One theory suggests that scleroderma is caused by a virus or other infection. For example, a common virus called CMV (cytomegalovirus) is known to infect cells (endothelial cells) that line our blood vessels, which we know are diseased in scleroderma (3).
How does scleroderma affect the kidneys?
In scleroderma renal crisis, some unknown event triggers blood vessel spasm and additional damage. This triggers aggressive high blood pressure which, in turn, leads to more kidney damage.
How is scleroderma renal crisis treated?
Blood pressure control with angiotensin-converting enzyme (ACE) inhibitors with gradual reduction of malignant hypertension is the cornerstone of treatment. Other agents such as calcium channel blocking agents may be added. Renal dialysis may even be required.
What fruits are good for scleroderma?
General Diet Recommendations:
| Food Group | Serving size |
|---|---|
| Fruit 2-3 servings per day | one small whole fruit 1 cup pieces (melon, grapes, berries) 1/4 dried (raisins, cherries) 1/2 cup canned |
| Vegetables 5-7 servings per day | 1 cup raw, chopped 1 cup leafy 1/2 cup cooked |
What is the root cause of scleroderma?
Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body’s connective tissues, including your skin. Doctors don’t know exactly what causes this process to begin, but the body’s immune system appears to play a role.
What is sclerosis of the kidney?
Glomerulosclerosis is scarring of the filtering part of the kidneys (glomerulus). This causes a loss of protein into the urine. These proteins help fluid stay within the blood vessels. Without them, fluid leaks into the nearby tissue causing swelling.
How is scleroderma renal crisis diagnosed?
How Scleroderma Renal Crisis Is Diagnosed. Patients with scleroderma will have their blood tested for antibodies, which can help confirm a diagnosis as well as help doctors understand more about the nature and severity of the disease. Anti-RNA-polymerase III antibodies are found in one-third of patients with SRC.
What foods make scleroderma worse?
Avoid foods that may aggra- vate symptoms such as citrus fruits, tomato products, greasy fried foods, coffee, garlic, onions, peppermint, gas-producing foods (such as raw peppers, beans, broccoli or raw onions), spicy foods, carbonated beverages and alcohol.