Scleroderma is a rare, progressive disease that leads to hardening and tightening of the skin and connective tissues. The term scleroderma literally means “hard skin,” which describes the skin from increased deposits of collagen.
Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Scleroderma results from an overproduction and accumulation of collagen in body tissues.
In addition to thickening and hardening of your skin, scleroderma can cause your skin to lose its elasticity and become shiny as it stretches across underlying bone.
It usually begins with a few dry patches of skin on the hands or face that begin getting thicker and harder. These patches then spread to other areas of the skin.
Scleroderma results from an overproduction of collagen, the primary connective tissue protein in the body. Scientists believe that the immune system mistakenly attacks the patient's own cells (an autoimmune response), producing too many white blood cells and other factors that cause a damaging inflammatory response and an overproduction of collagen, primarily in the skin.
Researchers are not clear why this autoimmune response occurs, but they suggest that both genetic and environmental factors play a role in the development of the disease. For example, scleroderma has been associated with a number of industrial and pharmaceutical chemicals including:
- Numbness, pain or color changes in your fingers, toes, cheeks, nose and ears, often brought on by cold or emotional distress (Raynaud's phenomenon)
- Stiffness or pain in your joints and curling of your fingers
- Digestive problems ranging from poor absorption of nutrients to delayed movement of food due to impaired muscular activity in your intestine.
- Sores over joints, such as your elbows and knuckles
- Puffy hands and feet, particularly in the morning
Scleroderma Facts and Statistics
- About 150,000 Americans have the disease.
- It's more common in women than in men and more common in adults than in children.
- Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease.
- The five-year survival rate is 80 to 85%.
Different Categories of Scleroderma
There are two main types of scleroderma with a subset called CREST:
1.Localized scleroderma affects the skin in limited areas and the musculoskeletal system.
2.Systemic sclerosis causes more widespread skin changes and may be associated with internal organ damage in the lungs, heart and kidneys.
Systemic sclerosis is associated with over-activation of the immune system, which normally functions to protect the body against cancers and invading infections. This causes damage to cells that line small blood vessels, which in turn leads to the over-production of scar tissue.
Systemic scleroderma may result in a number of other medical complications:
- Gastrointestinal complications: scleroderma can cause wasting in the muscular walls of your intestine reducing absorption of nutrients and movement within the intestine. This will lead to weight loss due to mal-absorption. Another complication of scleroderma is heartburn.
- Lung complications: difficulty in breathing can occur due to scarring in the lung tissue. This is commonly referred to as “pulmonary fibrosis. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
- Kidney complications: Scleroderma can affect the kidneys leading to an elevated blood pressure and an increased level of protein in your urine. More serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
- Heart complications: Scarring of heart tissue increases your risk of heart arrhythmias and congestive heart failure and can inflame the membranous sac surrounding your heart (pericarditis).
There is a subset of Systemic Sclerosis termed CREST
CREST is a form of Systemic Sclerosis (scleroderma) which is characterized by:
a) Calcinosis (calcium deposits), usually in the fingers.
b) Raynaud's Phenomen:numbness and coolness in the fingers, toes or nose in response to cold temperatures or stress.
c) Esophageal dysfunction: such as reflux or difficulty in swallowing.
d) Sclerodactyly:a tapering deformity of the bones of the Fingers.
e) Telangiectasia:small red spots on the skin of the fingers, face, or inside of the mouth.
It takes only two of the five CREST symptoms for a diagnosis of CREST to be made
A study performed at the University of Michigan Hospital using PABA potassium para-aminobenzoate) revealed that 90% of 224 patients treated with PABA experienced mild, moderate, or marked skin softening. The difference in skin softening attained by patients treated with PABA compared to that of patients who did not receive this medication was significant.
--Department of Internal Medicine, University of Michigan Medical School, Ann Arbor- 1988
The Diagnosis of Scleroderma
Scleroderma can be difficult to diagnose. It's rare and, early on, it can affect the skin as well as the joints, making it look like other diseases such as rheumatoid arthritis or lupus.
Diagnosis of scleroderma is based on clinical history and physical findings.
Laboratory, X-ray and pulmonary function tests determine the extent
and severity of internal organ involvement.
Common Tests Performed Include:
- High ESR or elevated ESR
- (Mildly/highly) elevated ANA levels
- Rule out Nanobacteria Infection
Researchers have identified tiny bacteria, known as nanobacteria, that may play
a role in the formation of calcification found in scleroderma and other
disorders. Cells infected by these bacteria develop deposits of the mineral
apatite on their walls.
- Rule Mycoplasma Infection
- Rule out Lyme Disease: Some studies reported an association between Borrelia burgdorferi , the infectious agent in Lyme Disease, and some cases of localized scleroderma patches).
- Parvovirus. In one study there was a higher prevalence of antibodies to a virus called parvovirus 19 in patients with systemic sclerosis than in patients without the disorder.
The Chemical Connection
Occupational exposure to certain chemicals are being investigated as a possible contributing factor to scleroderma.
These chemicals include:
- Silica
- Plastic materials such as epoxy resins and vinyl chloride
- Detergents
- Herbicides
- Organic solvents (such as trichloroethane, benzene, and carbon tetrachloride)
- Various drugs (such as bleomycin, amphetamine, cocaine, amfepramone, docetaxel, pentazocine, and penicillamine)
Additional Treatments for Symptoms of SclerodermaPulmonary Fibrosis
Studies are now reporting that cyclophosphamide (Cytoxan), an immunosuppressive agent, may be effective for preventing lung deterioration.
Kidney Complication and High Blood Pressure
Many medications are available for controlling blood pressure, but angiotensin converting enzyme (ACE) inhibitors appear to be the most effective for scleroderma patients. ACE inhibitors include captopril (Capoten), enalapril (Vasotec), quinapril (Accupril), benazepril, and lisinopril (Prinivil, Zestril).
Gastrointestinal Complications
Treatments for abnormalities in the esophagus are generally the same as those for gastroesophageal reflux (GERD) or heartburn.
Proton-pump or acid-pump inhibitors are the most common prescribed for GERD related to scleroderma. They work by inhibiting the so-called gastric acid pump that is required for the stomach's cells to secrete acid. The standard medications have been omeprazole (Prilosec),(Prevacid), pantoprozole (Protonix), esomeprazole (Nexium), and rabeprazole (Aciphex)
Skin Complications
A topical treatment, such as a moisturizer or corticosteroid medication that you apply to your skin. Corticosteroid medications impede your body's ability to make substances that can cause inflammation.
Light therapy (phototherapy) has also proved effective in treating the lesions that are associated with scleroderma, but more research is needed.
Alternative Treatments
At present, there are no proven treatments or cure for any forms of scleroderma. However, there are several "natural" things you can do for systemic scleroderma.
One thing which will give you the greatest odds of living longer with systemic scleroderma, is to monitor your blood pressure at home every day or two.
You should also ask your doctor to take your blood pressure on every office visit.
Stress can play havoc with your immune system, as well as trigger and worsen attacks of Raynaud's. Alternative therapies that help you deal more effectively with stress, such as biofeedback and yoga, may improve your symptoms.
Strive for moderation in everything - just enough but not too much exercise, good sleep every night with sufficient rest breaks during the day, and well-balanced, regular meals.
In general, most doctors recommend that scleroderma patients follow a normal, well-balanced diet, without the addition of any herbs, vitamins, or minerals.
Do not cut out any food groups from your diet, nor go on any "cleansing" diets, since this may accelerate the disease process.
For relief of symptoms such as Raynaud's and heartburn, you may want to particularly avoid:
- Caffeine (can trigger Raynaud's and heartburn)
- Chocolate (can trigger Raynaud's and heartburn)
- Peppermint (can trigger heartburn)
ginger (can trigger heartburn)
Supplements and Herbs can aggravate Scleroderma
Many herbs and over-the-counter medications can interact with prescription medications, and many of them make scleroderma symptoms worse. Even simple over-the-counter cold remedies often have ingredients that can trigger Raynaud's or heartburn in systemic scleroderma patients.
The immune system does not need to be improved in scleroderma, because it is already overactive. Doing things that enhance the immune system can actually make scleroderma worse in some cases, as it gives the body more power to attack itself.
Recommendations and treatments for Scleroderma:
DMSO and Skin Symptoms
Arthur Scherbel, MD, of the Department of Rheumatic Diseases and Pathology at the Cleveland Clinic Foundation, used DMSO on 42 scleroderma patients who had already exhausted all other possible therapies without relief. The investigators concluded that 26 of the 42 showed good or excellent improvement.
PABA & Sclerderma
The vitamin PABA (para-aminobenzoic acid) has been studied and found to have a postitive impact on Scleroderma.
Avocado/Soybean combination
A small study found that Avocado/Soybean combination may provide some relief for joint stiffness and pain.
References
Jablonska S.Avocado/soybean unsaponifiables in the treatment of scleroderma: comment on the article by Maheu et al.
Arthritis Rheum. 1998 Sep;41(9):1705.
Zarafonetis CJD. The treatment of scleroderma: results of potassium para-aminobenzoate therapy in 104 cases. In: Mills LC, Moyer JH eds. Inflammation and Diseases of Connective Tissue. Philadelphia: W. B. Saunders Co. 1961, 688–96.
Zarafonetis CJD, Dabich L, Negri D, et al. Retrospective studies in scleroderma: effect of potassium para-aminobenzoate on survival. J Clin Epidemiol 1988;41:193–205.
Zarafonetis CJ, Dabich L, Devol EB, et al. Retrospective studies in scleroderma: pulmonary findings and effect of potassium p-aminobenzoate on vital capacity. Respiration 1989;56:22–33.
