Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.
Scleroderma is a chronic condition that causes inflammation and
thickening of the skin. The cause of scleroderma is not known. Researchers have
found some evidence that genes are important factors, but the environment seems
to also play a role. This means that inheritance at least play a partial role.
It is not unusual to find other autoimmune diseases in families of scleroderma
patients. Some evidence for the role genes may play in leading to the
development of scleroderma comes from the study of Choctaw Native Americans who
are the group with the highest reported prevalence of the disease. The disease
is more frequent in females than in males.
A common area of involvement of this condition is
the skin over the tips of the elbows (the olecranon area). When this skin
is involved, it can lead to troublesome irritation of the tips of the elbows
with tenderness noted when any pressure is applied.
Recently, I saw a patient in a follow up visit who is a veterinarian with
scleroderma with this particular complication. We had been treating the
condition with a variety of topical lubricants and emollients, but to no avail.
She returned at this visit to tell me of a surprisingly successful treatment
that she had found on her own.
Scleroderma is an autoimmune disease of the connective tissue featuring skin thickening, spontaneous scarring, blood vessel disease, varying degrees of inflammation, associated with an overactive immune system. Autoimmune diseases are illnesses which occur when the body's tissues are attacked by its own immune system. Scleroderma is characterized by the formation of scar tissue (fibrosis) in the skin and organs of the body. This leads to thickness and firmness of involved areas. Scleroderma, when it's diffuse or widespread over the body, is also referred to as systemic sclerosis.
The cause of scleroderma is not known. Researchers have found some evidence that certain genes are important factors, but the environment seems to also play a role. The result is activation of the immune system in a susceptible individual, causing injury to tissues that result in injury similar to scar-tissue formation. The fact that genes seem to cause a predisposition to developing scleroderma means that inheritance at least plays a partial role. It is not
unusual to find other autoimmune diseases in families of scleroderma
patients. Some evidence for the role genes may play in leading to the development of scleroderma comes from the study of Choctaw Native Americans who are the group with the highest reported prevalence of the disease. The disease is more frequent in females than in males.
How is scleroderma classified?
Scleroderma can be classified in terms of the degree and location of the
skin involvement. Accordingly, scleroderma has been categorized into two
major groups, diffuse and limited. However, the terminology in the literature varies; for example, some investigators term the two major groups localized and systemic.
The diffuse form of scleroderma (systemic sclerosis) involves symmetric thickening of skin
of the extremities, face, and trunk (chest, back, abdomen, or flanks) that
can rapidly progress to hardening after an early inflammatory phase. Organ
disease can occur early on and be serious. Organs affected include the
esophagus, bowels, and lungs with scarring (fibrosis), heart, and kidneys.
High blood pressure can be a troublesome side effect.
The limited form of scleroderma tends to have far less skin involvement with skin thickening confined to the skin of the fingers and face. The skin changes and other features of disease tend to occur more slowly than in the diffuse form. Because a characteristic clinical pattern can occur in patients with the limited form of scleroderma, this form has taken another name that is composed of the first initials of the common components. Thus, this form is also called the "CREST" variant of scleroderma. CREST represents the following features:
C...Calcinosis refers to the formation of tiny deposits of calcium in
the skin. This is seen as hard, whitish areas in the superficial skin,
commonly overlying the elbows, knees, or fingers. These firm deposits can
be tender, can become infected, and can fall off spontaneously or require
surgical removal. This is the least common of the CREST scleroderma variant features.
R...Raynaud's phenomenon
refers to the spasm of the tiny artery vessels supplying blood to the
fingers, toes, nose, tongue, or ears. These areas turns blue, white, then
red after exposure to extremes of cold, or even sometimes with extremes of
heat or emotional upset. This can lead to tiny areas of damage to the tips of the fingers (digital ulcers) or larger areas of dead skin on the ends of the fingers.
E...Esophagus disease in scleroderma is characterized by poorly
functioning muscle of the lower two-thirds of the esophagus. This can lead to an
abnormally wide esophagus that allows stomach acid to backflow into the
esophagus to cause heartburn, inflammation, and potentially scarring that narrows the esophagus. This
can eventually lead to difficulty in passing food from the mouth through
the esophagus into the stomach. Symptoms of heartburn are treated
aggressively in patients with scleroderma in order to prevent injury to
the esophagus.
S...Sclerodactyly refers to the localized thickening and tightness of
the skin of the fingers or toes. This can give them a "shiny" and slightly puffy
appearance. The tightness can cause severe limitation of motion of the
fingers and toes. These skin changes generally progress much slower that
those of patients with the diffuse form of scleroderma.
T...Telangiectasias are tiny red areas, frequently on the face, hands,
and in the mouth behind the lips. These areas blanch when they are pressed
upon and represent widened (dilated) capillaries.
Patients can have variations of CREST, for example, CRST, REST, ST, etc.
Patients can also have "overlap" illness with features of both
CREST and the diffuse form of scleroderma. Some patients have overlaps of
scleroderma and other connective tissue diseases, such as rheumatoid
arthritis, systemic lupus erythematosus, and polymyositis. When features of scleroderma
are present along with features of polymyositis and systemic lupus erythematosus, the
condition is referred to as mixed connective tissue disease (MCTD).
Finally, scleroderma skin changes can be localized. Morphea is scleroderma
skin that is localized to a patchy area of the skin that becomes hardened and slightly
pigmented. Sometimes morphea can cause multiple lesions in the skin. Morphea is not associated with disease elsewhere in the body. Linear scleroderma
is scleroderma that is localized usually to a lower extremity, frequently presenting as
a strip of hardening skin down the leg of a child. Linear scleroderma in children can
stunt bone growth of the affected limb. Sometimes linear scleroderma is associated with a "satellite" area of a patch of localized scleroderma skin, such as on the abdomen.
High blood pressure, also known as hypertension, is a repeatedly
elevated blood pressure exceeding 140 over 90 mmHg -- a systolic pressure above
140 with a diastolic pressure above 90. There are two causes of high blood pressure, primary and secondary. Primary high blood pressure is much more common that secondary and its basic causes or underlying defects are not always known. It is known that a diet high in salt increases the risk for high blood pressure, as well as high cholesterol. Genetic factors are also a primary cause. Secondary high blood pressure is generally caused by another condition such as renal hypertension, tumors, and other conditions. Treatment for high blood pressure is generally lifestyle changes and if necessary, diet.
GERD (gastroesophageal reflux disease) is a condition in which the acidified liquid
contents of the stomach backs up into the esophagus. The symptoms of uncomplicated GERD are heartburn,
regurgitation, and nausea. Effective treatment is available for most patients with GERD.
Sjögren's syndrome is an autoimmune disease involving the abnormal production of extra antibodies that attack the glands and connective tissue. Sjögren's syndrome with gland inflammation (resulting dry eyes and mouth, etc.) that is not associated with another connective tissue disease is referred to as primary Sjögren's syndrome. Sjögren's syndrome that is also associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma, is referred to as secondary Sjögren's syndrome. Though there is no cure for Sjögren's syndrome, the symptoms may be treated by using lubricating eye ointments, drinking plenty of water, humidifying the air, and using glycerin swabs. Medications are also available to treat dry eye and dry mouth.
Raynaud's phenomenon is characterized by a pale-blue-red sequence of color changes of the digits, most commonly after exposure to cold. Occurring as a result of spasm of blood vessels, the cause is unknown. Symptoms of Raynaud's phenomenon depend on the severity, frequency, and duration of the blood vessel spasm. Treatments include protection of the digits, medications, and avoiding emotional stresses, smoking, cold temperature, and tools that vibrate the hands.
Small intestinal bacterial overgrowth (SIBO) refers to a condition in which abnormally large numbers of bacteria (at least 100,000 bacteria per ml of fluid) are present in the small intestine and the types of bacteria in the small intestine resemble more the bacteria of the colon than the small intestine. There are many conditions associated with small intestinal bacterial overgrowth, to include: diabetes, scleroderma, Crohn's disease, and others. There is a striking similarity between the symptoms of irritable bowel syndrome and SIBO. It has been theorized that SIBO may be responsible for the symptoms of at least some patients with irritable bowel syndrome. Symptoms of SIBO include: excess gas, abdominal bloating, diarrhea, and abdominal pain.
A keloid is a scar that doesn't know when to stop. When the cells keep on reproducing, the result is an overgrown (hypertrophic) scar or a keloid. A keloid looks shiny and is often dome-shaped, ranging in color from slightly pink to red. It feels hard and thick and is always raised above the surrounding skin.
Pulmonary fibrosis is scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes, infections, environmental agents, exposure to ionizing radiation, chronic conditions, and certain medications. Symptoms include shortness of breath, coughing, and diminished exercise tolerance. Treatment options are dependent on the type of pulmonary fibrosis; lung transplant and/or medications are optons.
Dysphagia or difficulty in swallowing, swallowing problems. Dysphagia is due to problems in nerve or muscle control. It is common, for example, after a stroke. Dysphagia compromises nutrition and hydration and may lead to aspiration pneumonia and dehydration.
Pulmonary hypertension is an abnormal elevation of the pressure in the pulmonary circulation caused by the constriction of the blood vessels that supply blood to the lungs. Shortness of breath and dizziness are symptoms of pulmonary hypertension. Treatment involves diuretics, blood thinners, calcium channel blockers, and using supplemental oxygen to increase blood oxygen levels.
Pericarditis is the inflammation of the pericardial sac that surrounds the heart. The causes of pericarditis include injury from heart attack, heart surgery, trauma; viral or fungal infection, HIV, tumors, mixed connective tissue disease, metabolic disease, medication reactions, or idiopathic. Treatment for pericarditis is generally medication, however, sometimes surgery is necessary.
Primary Biliary Cirrhosis is a chronic disease characterized by progressive inflammation and destruction of small bile ducts within the liver. The bile ducts transport bile from the liver to the intestine for the absorption of fat and elimination of waste products. The causes of Primary Biliary Cirrhosis may involve autoimmunity, infection, or genetic predisposition, acting alone or in combination. There are many medications and treatment options available for those with this and other associated diseases of Primary Biliary Cirrhosis.
Interstitial lung disease, is a term to describe a certain lung condition. Causes of interstitial lung disease include lung infection, exposure to toxins in the environment (asbestos for example), medications (chemotherapy), radiation therapy, and chronic autoimmune disorders. Common symptoms of interstitial lung disease include a dry cough and shortness of breath. Diagnosis and treatment depend upon the cause of the condition.
Polymyositis is a disease of the muscle featuring inflammation of the muscle fibers. It results in weakness of the muscles which can be severe and when associated with skin rash, is referred to as dermatomyositis. Although the cause of this disease is unknown, diagnosis includes physical examination of muscle strength, blood tests for muscle enzymes, electrical tests of muscle and nerves, and conformation by a muscle biopsy. Treatment of Polymyositis and Dermatomyositis includes high doses of cortisone-related medications, immune suppression, and physical therapy.
Connective tissue disease is when the body's connective tissues come under attack, possibly becoming injured by inflammation. Inherited connective tissue diseases include Marfan syndrome and Ehlers-Danlos syndrome. Systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymositis, and dermatomyositis are examples of connective tissue diseases that have no known cause.
Connective tissue diseases are disorders featuring abnormalities involving the collagen and elastin.
Connective tissue diseases that are strictly inheritable include Marfan syndrome and Ehlers-Danlos syndrome. The classic immune-related connective tissue diseases include systemic lupus erythematosus, rheumatoid arthritis, scleroderma, polymyositis, and dermatomyositis. Treatment is often directed at suppressing the inflammation present in the tissues by using anti-inflammatory and immunosuppressive medications.
Preeclampsia is related to increased blood pressure and protein in the mother's urine. Preeclampsia typically begins after the 20th week of pregnancy. When preeclampsia causes seizures, it is termed "eclampsia" and is the second leading cause of maternal death of in the US. Preeclampsia is the leading cause of fetal complications. Risk factors for preeclampsia include high blood pressure, obesity, multiple births, and women with preexisting medical conditions such as diabetes, kidney disease, rheumatoid arthritis, lupus, or scleroderma. Pregnancy planning and lifestyle changes may reduce the risk of preeclampsia during pregnancy.
Bowel or fecal incontinence refers to the loss of voluntary control of stool, or bowel movements. The condition can include partial incontinence, in which a person loses only a small amount of liquid waste, to complete incontinence, in which the entire bowel movement cannot be controlled. Diet changes and elimination of certain medications can help patients to regain bowel control. Treatment involves a combination of medication, biofeedback, and exercise.
Eosinophilic fasciitis is a skin disease that causes thickening and inflammation of the skin and fascia. Symptoms include redness, warmth, and hardening of the skin, as well as occasional tissue and joint pain. Treatment for eosinophilic fasciitis aims to eliminate inflammation through the use of aspirin, NSAIDs, and cortisone. Aggressive forms of eosinophilic fasciitis may require the use of immune suppression medications.
Eosinophils are a particular type of white blood
cells, usually representing a small percentage (less than 8% of
the total white blood cell population). The number
of these cells (eosinophil count) increases in certain illnesses, including allergies,
asthma, Addison's disease, sarcoidosis, parasite infections, drug
reactions, and connective tissue diseases (such as rheumatoid
arthritis and scleroderma).
What is fascia?
The fascia is a lining tissue under the skin that covers a
surface of underlying tissues. When the fascia is inflamed, the condition is
referred to as "fasciitis."
What is eosinophilic fasciitis?
Eosinophilic fasciitis is a specific disease of the skin that leads to
inflammation and thickening of the skin and fascia underneath.
In patients with eosinophilic fasciitis, the involved fascia ...