Rheumatic Skin Disease: Frequently Asked Questions

Why are there skin problems that occur in rheumatoid patients?
Almost all of the diseases in this category are thought to be diseases in which one's immune system is not working properly. The immune system is a normal part of one's body that is designed to protect us from infections with germs and viruses from the environment. Our immune system also helps us ward off cancer cell development in our bodies. When one's immune system gets out of control and starts attacking one's own bodily tissues, this is called "autoimmunity." In this situation, blood proteins called autoantibodies are produced that bind to and injure one's own bodily tissues. Rheumatic diseases such as rheumatoid arthritis, lupus, dermatomyositis, and scleroderma are thought to be autoimmune diseases.

In a disease like rheumatoid arthritis, the immune system abnormalities can attack the lining of the joints. This produces arthritis or inflammation in the joints. (The term "inflammation" means a combination of pain, tenderness, swelling, and redness.) The same type of immunological abnormalities that occur in the joint of a rheumatoid arthritis patient also can occur in the skin. As a result, we can see skin lesions in patients with rheumatoid arthritis that can reflect the state of activity of the immunological abnormalities inside of that patient.
What do the following tests mean: ANA, C&P ANCA, LP, and CFS?
There are some blood tests one can do that can reflect how active the immunological disease is inside our bodies in diseases like rheumatoid arthritis, lupus, and scleroderma. These tests are routinely ordered by doctors who are treating such patients. This information can be very helpful at times both in the diagnosis of the specific problem and in guiding treatment of the problems over time. In a disease like rheumatoid arthritis, the most common blood test abnormalities are the presence of rheumatoid factor. Rheumatoid factor is a type of autoantibody present in the blood of almost all patients with rheumatoid arthritis who have the really destructive form of the disease. This particular test also indicates a risk for some of the complications that can occur in rheumatoid arthritis, in parts of the body outside of the joints. A test like the rheumatoid factor certainly can be helpful in making the initial diagnosis of rheumatoid arthritis. In addition, the amount of rheumatoid factor in the blood can be an indication of the state of activity of the immunological illness inside the body.

Another test is the antinuclear antibody assay (the "ANA test" for short). The ANA test is almost always positive in rheumatic diseases such as lupus. In addition, it is often positive in rheumatoid arthritis, dermatomyositis, and scleroderma. This test, since it can be positive in a number of these diseases, is not diagnostic of any one particular disease. A physician will use the ANA test to screen for this general group of illnesses, and if that is positive, then will do more specific tests to make a specific diagnosis such as lupus. However, one thing that must be kept in mind in interpreting the results of the ANA test is that it can also be positive in other disease settings that are not related to arthritis. Even normal individuals, on occasion, will have abnormal ANA test results. This occurs even more frequently in older, healthy individuals. Certain medications can trigger a positive ANA test. The point is that the physician must be very careful in interpreting the results of the ANA test and should counsel patients about the true meaning of an ANA test result. I see a lot of confusion produced as a result of physicians in the community not being fully aware of the various pluses and minuses of the ANA test.

I will mention one other laboratory test in this context. That is the ANCA test. ANCA stands for "antibody to neutrophil cytoplasmic antigens." This test is often positive in forms of blood vessel inflammation such as vasculitis. One of the strongest disease associations of the ANCA test is a disease called Wegener's granulomatosis. This is a disease that can attack blood vessels in different parts of the body, including the skin. Recognizing the characteristic patterns of skin changes can be a clue to the diagnosis of this disease and getting patients on proper treatment for the internal complications that can be very severe (lung and kidney injury). However, like the ANA test, one must be careful in interpreting the ANCA test results. The ANCA test can be positive in other conditions besides vasculitic illnesses, such as Wegener's granulomatosis.
How are these skin conditions commonly treated?
The answer to this question is complex. The skin lesions in a large number of the 100 or so diseases that cause arthritis are treated differently. For example, in a disease like lupus, the skin lesions can be treated quite nicely with cortisone-containing creams and oral medications such as the antimalarials. However, these same forms of treatment usually do not help the skin changes that we see in scleroderma. The treatment really has to be individualized to the specific disease and to the specific conditions related to a given case. Some drugs might be riskier in women compared to men, for example.
What about the following tests: Ro and La antibody titers?
The Ro and La antibody tests give information somewhat similar to the results of an ANA test. However, the Ro and La antibodies are specific autoantibodies that can reflect a risk for certain types of rheumatic disease. For example, the Ro antibody test, when positive, can indicate a risk for certain forms of lupus skin disease, such as subacute cutaneous lupus erythematosus and neonatal lupus erythematosus. In addition, Ro antibodies also can indicate risk for Sjogren's syndrome.

Subacute cutaneous lupus erythematosus (also known in its abbreviated form as SCLE) is a form of lupus skin disease that is made worse by exposure to sunlight or artificial sources of ultraviolet radiation and does not produce scarring. It produces scaly red patches on the skin that can simulate the appearance of psoriasis occurring in sun-exposed areas of the body. Patients with this form of skin lupus have a somewhat higher risk for developing the more severe internal complications of systemic lupus erythematosus compared to another common form of lupus skin disease named discoid lupus erythematosus. Discoid lupus erythematosus (also referred to as DLE) produces scaly coin-shaped lesions most commonly occurring on the face or scalp, although other parts of the body can be affected. This type of skin lupus often produces scarring of the skin and hair loss that can be permanent. In addition, discoid lupus skin lesions often produce darkening and/or lightening of the skin color. When lupus shows itself initially only as discoid lupus skin lesions, such patients are at very low risk for later developing serious internal problems from systemic lupus.

Neonatal lupus is a condition in which newborn babies develop skin lesions often simulating the appearance of subacute cutaneous lupus erythematosus. However, this occurs only when the mother of the newborn baby also had an immunological abnormality during pregnancy that resulted in her body producing Ro autoantibodies. It is thought that the mother's Ro autoantibodies cross over into the baby's blood circulation while still in the womb. These autoantibodies appear to be actually causing the skin lesions that occur after the baby is born and exposed to sunlight and other forms of ultraviolet light. Normally neonatal LE is a mild condition and the skin lesions go away on their own as the child gets older when the autoantibodies from the mother's blood disappear from the baby's blood. However, there is another complication that can occur in this setting and that is congenital heart block. Rather than developing skin lesions after delivery, babies develop damage in the conduction system in their hearts while still in the womb. This can be a very severe complication requiring permanent pacemaker placement in the heart of the baby.

The third condition I mentioned that is associated with Ro antibodies is Sjogren's syndrome. This is a condition that produces dryness in the eyes and mouth, most commonly in adult women. This dryness results from autoimmune damage to the glands that make tears and saliva. This is one of the most common of all rheumatic or arthritis-associated diseases but among the most difficult to diagnose–it is very often not diagnosed until it is quite advanced. Patients who have Sjogren's syndrome also experience body tenderness and lethargy that can simulate conditions such as fibromyalgia. In addition, internal organ damage can occur in Sjogren's, such as kidney problems, nerve injury, and blood vessel injury.

I have not said anything so far about the La autoantibody; however, everything I have said so about the diseases that are associated with Ro autoantibodies also applies to the La autoantibody. These two autoantibodies almost always occur as a pair. However, the Ro autoantibody occurs more frequently than the La autoantibody.
What is an LP test? A doctor I am seeing asked if I had an LP and if so what does the CSF show–can you explain?
An LP test, at least as that term is used most often, refers to a lumbar puncture test. This is a test where a needle is placed through the lower part of the back between the backbones and into the spinal column. However, the needle is positioned where it does not actually touch the spinal cord. Fluid, called cerebral spinal fluid, that normally circulates around the spinal cord can be withdrawn through this hollow needle. Various types of tests can be done on this sample of cerebral spinal fluid. Abnormalities in these tests can reflect brain and spinal column involvement with diseases like systemic lupus. Other diseases such as meningitis (bacterial [germ] infection of the membranes that cover and protect the brain and spinal column) can also cause abnormalities in the LP test.
Who is most affected by these conditions, men or women, and is there an age group more at risk?
The group of diseases that we call "rheumatic diseases" affect women more often by far. I might also mention that another term that is used to describe this group of diseases is the "autoimmune connective tissue diseases." Another term that has been used in the past to describe these diseases is "collagen vascular diseases." It is not fully understood why women have a greater risk for developing rheumatic diseases and their associated immunological abnormalities. However, there is some evidence in experimental animal models of diseases like systemic lupus that indicate that female sex hormones, such as estrogen, can potentiate or aggravate the immunological disturbances of lupus. That information has led to some concern about patients with immune diseases like lupus taking drug forms of estrogen such as birth control pills or Premarin. However, the studies that have been done to date would indicate that if there is a risk from such treatment, it would appear to be relatively mild.
What are the symptoms of these skin conditions, and how can you distinguish whether they are caused by rheumatoid-related illness and not something else?
Again, the answer to this question is very complex. I will try to keep it as straightforward as possible. Recall that there are as many as 100 different medical conditions that can be associated with joint inflammation or arthritis. All of these conditions are different and seem to represent unique abnormalities in the human body. Therefore, one would expect that the skin problems that might occur in these many different disorders would be different and would require different forms of treatment. However, let me focus on a couple of the major illnesses in this area and describe how the skin lesions occur and how they are treated in general.

In rheumatoid arthritis, one of the more common ways that the skin is affected is through inflammation in the walls of the blood vessels of the skin producing a condition called vasculitis. Since vasculitis also can occur in internal tissues, like nerves, in rheumatoid arthritis it is necessary to treat patients having this type of skin problem with drugs by mouth or by vein that can dampen down the autoimmune abnormalities that are producing the inflammation in the blood vessel walls.

Some drugs like the corticosteroids ("steroids") suppress the immune response in a broad fashion and can be very useful in a number of autoimmune diseases, including the rheumatic diseases (corticosteroids are commonly referred to as "cortisone" type drugs). Corticosteroids like prednisone taken by mouth can certainly suppress various manifestations of rheumatoid arthritis, including the skin changes like vasculitis. However, longterm use of corticosteroids by mouth can produce a lot of troublesome and serious side effects. Therefore, physicians are constantly trying to find other drugs that will prevent having to rely on corticosteroids for long periods of time. In the case of rheumatoid arthritis, methotrexate is a drug that has been found to be able to prevent patients from having to take so much corticosteroid. Generally, the things a dermatologist does to treat the surface of the skin, such as applying sunscreens and cortisosteroid-containing creams or ointments, does not help the more severe skin problems such as vasculitis that are seen in rheumatoid arthritis.

Another common autoimmune rheumatic disease in which the skin is often injured is lupus erythematosus. There are a number of different types of skin changes that can be seen in these patients. Interestingly, these different types of skin lesions can provide different types of information about the risk of a patient developing the more severe internal complications of lupus. For example, the presence of discoid lupus erythematosus skin lesions at the beginning of a patient's illness generally indicates a lower risk for developing the severe internal complications of systemic lupus such as kidney damage or brain damage. However, other forms of lupus skin disease, such as the red butterfly-shaped rash that occurs on the cheeks and nose of the face and is often triggered by exposure to sunlight, is generally seen in patients who have a higher risk for developing severe internal complications of systemic lupus. Recognizing the pattern of skin damage that occurs in patients with lupus can have the additional benefit of helping to predict the severity of illness that a given patient might experience over time.

Regarding treatment of lupus skin disease, the topical measures that were discussed above, such as the application of sunscreens and corticosteroid-containing creams and ointments directly to the skin, can be helpful in suppressing the skin inflammation caused by lupus. However, most skin lupus patients do require some type of oral therapy in addition to the topical therapy. The safest form of oral therapy to treat lupus skin disease would be one, or a combination, of the anti-malarial drugs, such as hydroxychloroquine, which is commonly referred to by its trade name: Plaquenil. This drug can be used very safely if the common recommended guidelines concerning total daily dosage are followed. However, patients need to have their eyes monitored while on this drug since on rare occasion problems can develop in the retina of the eye while on this form of treatment.

Similar forms of treatment are used for the skin problems seen in patients with dermatomyositis. This condition causes autoimmune inflammation and damage in the muscles, skin, and occasionally other vital organs, such as the lungs. However, dermatomyositis skin disease generally is harder to treat than is lupus skin disease. In addition, dermatomyositis skin disease is often more troublesome for the patient by producing symptoms such as itching (lupus skin disease usually does not itch).

The final condition that I will comment upon in this regard is scleroderma. Scleroderma is a term that just means 'hard skin.' Like lupus, patients having scleroderma skin changes have a variable risk for having associated damage to internal organs, especially the kidneys and lungs. Some patients develop a form of scleroderma that never goes on to cause damage to internal organs. This form of the disease is called localized scleroderma or morphea. However, other patients with scleroderma do develop internal complications relatively soon after the onset of skin problems. I am often asked how one can distinguish between these two forms of scleroderma at the bedside. A simple rule of thumb is that patients having scleroderma who are also at risk for developing internal complications usually have their disease start with Raynaud's phenomenon, whereas, patients with the localized form of scleroderma or morphea that only causes skin problems do not develop Raynaud's phenomenon. Raynaud's phenomenon is a condition resulting from temporary decrease in the flow of blood to the fingertips and toe tips, and sometimes even the tips of the ears and tip of the nose. This decreased blood flow pattern often is triggered by exposure to cold temperatures or when experiencing stressful situations. Patients often initially notice pain in their fingertips, followed by discoloration in those areas. The classical combination of color changes in Raynaud's phenomenon is initially a blanching or whitening of the fingertips, followed by a bluish discoloration of the fingertips, followed by redness in these areas.
What is a sed rate test, and what is the reference range for testing?
The sed rate is a non-specific test that, when abnormal, indicates generalized inflammation somewhere in the body from virtually any cause. The test is done by measuring the rate of sedimentation (or falling) of the red blood cells to the bottom of a tube when a sample of blood is placed in an upright position. The rate at which the blood cells fall is a general reflection of protein changes in the blood that occur as a result of inflammation in the body. Of course, inflammation is present in many of the arthritis-associated diseases since arthritis literally means "inflammation in the joints." The sed rate can be done in one of several ways. However, values lower than 25 mm per hour generally do not reflect significant systemic inflammation.

Some rheumatic diseases produce greater abnormalities in the sed rate than others. In patients having systemic lupus that is very active internally, the sed rate can be very high, 50 mm and above. Another rheumatic disease in which very high sed rates can be seen, sometimes above 100 mm per hour, is a condition called "temporal arteritis" that is often associated with another condition called "polymyalgia rheumatica." These patients have a condition that causes inflammation in the walls of the blood vessels, particularly the larger arteries near the surface of the skin in the temporal areas of the forehead and scalp. Damage to these blood vessels generally shows itself initially as very severe headaches. Overt skin changes occur only occasionally in this condition and, when present, generally consist of ulceration of skin of the scalp. Patients who have temporal arteritis can develop a great deal of pain and stiffness in their shoulders and upper trunk as a result of the associated condition polymyalgia rheumatica. It is very important that temporal arteritis is recognized early since delay in treatment can result in devastating complications such as stroke or blockage of the artery that supplies the retina layer in the eye, resulting in blindness.