Skin Disease In Dermatomyositis
-- What Patients And Their Families Should Know --
content prepared by
Richard D. Sontheimer M.D.
John S. Strauss Endowed Chair in Dermatology
Professor and Head, Department of Dermatology
University of Iowa College of Medicine
Edited for readability by
Dianna Geers, B.A.
Last Update: Sept. 3, 2000
This information will be presented as answers to "frequency asked questions" about skin disease in dermatomyositis.
Brief answer:
Dermatomyositis is an autoimmune disease that damages the skin and muscles resulting in a characteristic rash and weakness. The rash has a unique appearance and distribution over the body. The muscle weakness is most prominent in the shoulders and hips. Some patients initially develop the skin rash but never experience muscle weakness (clinically-amyopathic dermatomyositis).
More complete answer:
Dermatomyositis is an autoimmune disease that causes damage to the skin (i.e., dermato) and certain types of muscles (i.e., myo). (See glossary for definition of terms such as "autoimmune.") There are two forms of dermatomyositis - the classical form and the clinically-amyopathic form ("a" – meaning no or without, "myopathic" meaning muscle disease.
The classical form of dermatomyositis consists of a characteristic skin rash and muscle weakness most often affecting the shoulders and hip muscles. In the clinically-amyopathic form of dermatomyositis, the skin rash can be present for long periods of time (six months or longer) without the appearance of muscle weakness.
The rash of dermatomyositis initially appears as a red-purple, itchy skin change. This rash is most prominent on the upper eyelids, the cheeks of the face, the outside or hairy surface of the arms, the backs of the hands and fingers, elbows, hips, knees, and ankles. Other areas of the skin that can be affected with the itchy red-purple skin rash include the scalp, the V area of the front part of the neck and upper chest where the collar is open, over the back of the neck and shoulders, the mid and lower back and the bottom (buttocks). One or a combination of these areas can be affected in a given patient.
The dermatomyositis rash is often very itchy and can be associated with scaling or flaking of the skin (hyperkeratosis). The rash characteristically occurs on the skin overlying the knuckles of the hands and the skin around the fingernails. Small red bumps can develop over the outside (lateral) parts of the knuckles. These are called Gottron's papules, named after the German doctor who first described them in the 1930s.
The red-purple rash can appear in the skin that borders the fingernails. Small but visible blood vessels appearing as fine red lines that are perpendicular to the junction of the skin around the nail and the nail plate (hard portion of the nail) can be seen in this area. In addition, the cuticle of the fingernails often gets very ragged and irregular.
Occasionally, the rash of dermatomyositis can be so severe that blisters and/or ulcers can form in the skin. In the late stages of the dermatomyositis skin rash, small hard lumps can develop under the surface of the skin due to the presence of calcium deposits. These can feel like pebbles or small stones under the skin's surface.
The first way that patients notice that their muscles are being affected by dermatomyositis is pain and/or weakness. This is usually first noted in the shoulder and hip muscles. Activities such as attempting to raise the arms above the head to comb the hair or to remove dishes from a kitchen cabinet on the wall become difficult to do. Patients also frequently find it difficult to rise out of a chair from a sitting position without using one's arms to help push oneself up. Also, it can be difficult to arise when one stoops over to pick something up off the floor.
In addition to weakness in the shoulder and hip muscles, pain and tenderness can also develop in these muscles. In some patients, muscle pain and tenderness appears before weakness develops. It is not unusual for the typical dermatomyositis rash to develop several weeks or months before an individual notices muscle weakness or other symptoms of dermatomyositis muscle disease. Patients are classified as having clinically-amyopathic dermatomyositis when muscle weakness has not developed within six months after onset of the typical dermatomyositis rash.
The muscles of the upper part of the swallowing tube (esophagus) that connects the mouth to the stomach can also be damaged by dermatomyositis. This can make it difficult to begin or start a swallow and can produce the sensation of a choking sensation. In very severe cases the muscles in the chest responsible for breathing can also be affected by dermatomyositis. However, only a small percentage of patients develop this particularly severe symptom.
Brief answer:
When the skin rash of dermatomyositis has fully developed, it can be diagnosed by its appearance alone. A small skin biopsy can be carried out to confirm the diagnosis. A number of other conditions can cause muscle weakness. Therefore, diagnosing dermatomyositis as the cause of muscle weakness can be more challenging. A combination of muscle strength testing, blood tests, tests of muscle electrical activity, radiologic (x-ray) imaging of muscles, and muscle biopsy is often required to confirm the diagnosis of dermatomyositis as the cause of muscle weakness.
More complete answer:
When the skin rash of dermatomyositis has fully developed, it can be diagnosed by its appearance alone. A small skin biopsy can be carried out to confirm the diagnosis. Diagnosing dermatomyositis as the cause of muscle weakness can be more challenging.
In addition to the characteristic skin rash and muscle weakness, certain blood tests are often abnormal in patients with classical dermatomyositis. The antinuclear antibody test (ANA) is often positive in patients with both classical dermatomyositis and clinically-amyopathic dermatomyositis. In addition, in classical dermatomyositis there are elevated blood levels of muscle enzymes such as creatine kinase and aldolase (these are chemicals that leak into the blood from damaged muscles). However, in clinically-amyopathic dermatomyositis patients, the blood levels of these muscle enzymes are normal.
Several muscle tests are frequently ordered by physicians when they suspect a diagnosis of classic dermatomyositis. Examples include an electromyogram which is a test that measures the electrical activity of muscles. This test requires multiple tiny needles being placed superficially in the skin. The characteristic electromyographic changes of dermatomyositis can be diagnostic of dermatomyositis muscle damage.
Muscle biopsy is also carried out so that muscle tissue can be examined under the microscope by a pathologist to identify the diagnostic changes that can be seen in the muscles in this disease. A muscle biopsy is done by surgically removing a small piece of muscle tissue under local (awake) or general (asleep) anesthesia.
Imaging studies such as magnetic resonance imaging (MRI), muscle spectroscopy and muscle ultrasonography are being used more and more. These are noninvasive tests (noninvasive means that needles or surgery is not required). However, these tests are not as diagnostic as electromyography or muscle biopsy. The noninvasive tests are increasingly being used to identify damaged muscles prior carrying out invasive tests such as muscle biopsy. Muscle damage from dermatomyositis can be quite spotty within a given muscle group. Directing the biopsy to muscles that look abnormal on one or more of these noninvasive muscle imaging procedures can increase the diagnostic accuracy of muscle biopsy.
Brief answer:
The brief answer to this question is that we do not know. However, scientists feel that a set of predisposing genes inherited from one’s parents is required. When exposed to certain viral infections or chemicals, individuals who are genetically predisposed tend to develop autoimmune reactions that damage the skin and muscle tissue in a characteristic manner.
More complete answer:
The exact answer to this question is not known. However, it appears that the skin and muscle inflammation that are seen in classic dermatomyositis are caused by autoimmune reactions. Autoimmunity occurs when one's body loses control of its own immune system (see glossary for further explanation of the immune system). The presence of autoantibodies in the blood of dermatomyositis is one of the reasons that faulty immune responses are thought to play a role in dermatomyositis. Antinuclear antibodies bind to chemicals normally present inside one's own normal muscle cells. (See the glossary for further discussion of immunologic terms such as "autoantibodies").
It is thought that environmental events such as certain viral infections or chemicals can trigger off these abnormal immune responses that end up damaging one's own skin and muscle tissue. One example is the HIV virus which can produce dermatomyositis-like symptoms in patients with AIDS. Other virus infections have also been implicated such as the Coxsackie virus.
However, infection alone does not appear to be the only cause of the faulty immune responses that produce dermatomyositis. One's particular set of genetic traits also appear to be important. If one inherits the combination of genes that predisposes to dermatomyositis from one's parents, then one is susceptibility to developing dermatomyositis when one is exposed to triggering virus infections or chemicals. Research studies are currently being carried out to identify which particular genes are responsible for causing the abnormal immune responses that appears to be responsible for dermatomyositis.
Some scientists believe that certain viruses that can infect muscle cells can change certain substances that normally reside within these cells in a way that become the focus of an attack by one's own immune system. Research is being done in this area to better understand how autoantibodies that bind to muscle substances are formed and what role they might play in damaging muscle and skin tissue in dermatomyositis. In addition, similar work is being carried out to try to understand if components of the immune system other than autoantibodies such as T-lymphocytes might also be capable of attacking one's own skin and muscle cells in dermatomyositis.
Classical dermatomyositis patients are also at risk for certain internal medical complications. One such problem is the development of cancers inside the body. Women with dermatomyositis most commonly develop ovarian, breast, uterine, colon, rectum, and lung cancers. Men more often develop lung, colon, rectum, testicular cancers. Older individuals (> 50 years of age) and those with a great deal of skin rash are at the greatest risk for developing an internal cancer. In this setting, approximately 1 in 4 classical dermatomyositis patients develop some type of internal cancer. It is not yet known if patients with clinically-amyopathic dermatomyositis also have a significantly increased risk of developing internal cancers.
It is thought that this increased risk of developing internal cancers disappears approximately two years after a patient is diagnosed as having classic dermatomyositis. During that two year period, dermatomyositis patients should carefully observed for the development of any signs of cancer. In a number of patients when the internal cancers have been successfully treated, the dermatomyositis symptoms markedly improve or disappear.
Patients with classic dermatomyositis also have an increased risk for inflammatory damage to their lung tissues that can produce severe shortness of breath and at times, death. This complication is referred to as interstitial pneumonia and has been the cause of death in clinically-amyopathic dermatomyositis patients.
Brief answer:
Very little, since no formal research studies have been carried out to date on this form of dermatomyositis. Our current understanding on this type of dermatomyositis that is described below results from a series of medical case reports only.
More complete answer:
This is a rare form of dermatomyositis in which the very same characteristic skin changes that occur in patients with classical dermatomyositis described above occur in individuals for prolonged periods of time (greater than six months) without that individual developing muscle weakness of any sort. In addition, such an individual by definition also has normal blood levels of muscle enzymes such as creatine kinase and aldolase.
Clinically-amyopathic dermatomyositis is a very poorly understood subgroup of dermatomyositis patients. It is not known whether such patients truly have an increased risk of complications that can be seen in patients with the classical form of dermatomyositis (e.g., internal cancer formation, interstitial pneumonia). Some patients with clinically-amyopathic dermatomyositis have been observed to have the characteristic skin changes of dermatomyositis for 20 years or longer without developing any signs of muscle weakness whatsoever. However, other clinically-amyopathic dermatomyositis patients have been observed to develop muscle weakness after having only the skin changes of dermatomyositis for 3-4 years.
Brief answer:
Classical dermatomyositis can develop at any age and in both sexes. Clinically-amyopathic dermatomyositis occurs more often in females DM. Some viral infections are thought to be trigger factors for dermatomyositis. The specific genes that predispose one to dermatomyositis have yet to be identified.
More complete answer:
Dermatomyositis can occur in both sexes and at any age. Women are somewhat more likely to develop the classical form of dermatomyositis compared to men. The current evidence suggests that women in the USA are at even greater of developing clinically-amyopathic dermatomyositis compared to men.
Both the classical and the clinically-amyopathic dermatomyositis forms of dermatomyositis can develop in adults and children. The juvenile (or childhood) form of dermatomyositis, by definition, occurs in individuals less than 18 years of age. Juvenile-onset dermatomyositis differs in several ways from the adult-onset form. The juvenile-onset form is not associated with a significantly increased risk for developing internal cancers as is the adult form. However, children who develop the classical form of dermatomyositis have a higher complication rate from damage to internal blood vessels. This can result in catastrophic complications such as death of a segment of the bowel in the abdomen. In addition, damage to the retinal layer of the eye can result in blindness. Also, there is a greater risk for developing calcium deposits in damaged tissue with associated complications such as overlying skin ulceration and infection. However, children who develop dermatomyositis are not at increased risk for developing internal cancers.
It has recently been observed that the juvenile-onset form of dermatomyositis can also present as clinically-amyopathic dermatomyositis. That is, children can have only the skin changes of dermatomyositis for abnormally prolonged periods of time without suffering from muscle weakness or other systemic complications of dermatomyositis.
As previously discussed, it is thought that individuals who develop all forms of dermatomyositis including the juvenile-onset form do so as a result of a genetic predisposition. Such individuals have inherited combinations of abnormal genes from their parents that allows for faulty immunological responses to develop in the skin and muscle tissue.
Brief answer:
The very earliest symptom is usually a red-purple skin change that is often quite itchy. The first areas of the skin to be affected can be that on the upper eyelids, the scalp, the cheeks of the face, the outer hairy aspects of the arms and forearms, the backs of the hands and fingers. Red-purple skin changes overlying the skin of the knuckles, around the fingernails, the elbows, the knees, and the V area of the neck and upper chest can also be present early on. If the skin rash is very severe blisters and ulcers can develop. Longstanding skin disease can result in a combination of thin skin associated with dark and light colored skin color parches of skin and visible blood vessels (poikiloderma).
More complete answer:
The very earliest symptom is usually a red-purple skin change that is quite itchy. The first areas of the skin to be affected can be that overlying the upper eyelids, the scalp, the cheeks of the face, the outer hairy aspects of the arms and forearms, the backs of the hands and fingers. Red-purple skin changes overlying the skin of the knuckles, around the fingernails, the elbows, the knees, and the V area of the neck and upper chest can also be present early on. In many patients, especially those with classical dermatomyositis, once the rash appears in these areas, it tends to slowly extend to involve larger areas of the skin and becomes even more itchy. After a while, a fine scale can develop over skin that has been affected by dermatomyositis. In addition, small scaly bumps can appear around the base of the hairs on the outer aspects of the arms, forearms, hips and thighs.
Skin involvement in some severely-affected patients can progress to a point where most of the skin over the body is affected with a very itchy, red-purple rash. The itching can be so severe as to cause sleep deprivation and occupational as well as psycho-social disability. Also, in extreme cases, blisters can form in dermatomyositis skin lesions. When these blisters break down, they can be very slow to heal and sometimes can produce ulcers in the skin. After long periods of dermatomyositis skin inflammation, calcium deposits can develop under the surface of the skin producing hard stone-like lumps. This occurs most frequently around the elbows, forearms, and hands.
Some patients with clinically-amyopathic dermatomyositis can have one or more abnormal muscle test although they continue to have no muscle weakness. The term "hypomyopathic dermatomyositis" has recently been used to refer to such patients. It should be emphasized that the skin changes that occur in patients with the classical form of dermatomyositis and the clinically-amyopathic form of dermatomyositis are thought to be identical in appearance and under the microscope.
Brief answer:
Yes. But in lost patients they will return after treatment is stopped.
More complete answer:
The answer to this question is "yes." Some patients develop dermatomyositis skin disease and have the activity of their dermatomyositis completely controlled by medical treatment. Such patients might never have subsequent episodes of skin disease activity even when their treatment is withdrawn. However, the more common outcome for dermatomyositis skin disease is that it returns to some degree at some point in time after it has initially been suppressed with treatment.
In some patients with the classical form of dermatomyositis, skin disease activity returns while the muscles remain normal. Thus, some patients that initially have the dermatomyositis rash and muscle weakness, redevelop only the skin rash when their disease activity returns following withdrawal of treatment. In this setting, the skin rash can be the patient's main day-by-day medical problem. The skin rash in this situation can be very difficult to further suppress with treatment. Such patients often require some form of systemic treatment to suppress the overactivity of the immune system. Such systemic treatments can be given by mouth (oral) or by needle injection (intramuscular or intravenous).
When skin disease activity smolders on in the same area of skin for long periods of time, the affected area of skin can take on a different appearance that is referred to as "poikiloderma." Poikiloderma is one of those fancy dermatological terms that indicates the simultaneous presence of four changes in the skin: hyperpigmentation (darkly colored skin spots), hypopigmentation (lightly colored skin spots), atrophy (thinning), and telangiectasia (the appearance of tiny blood vessels that are visible to the naked eye). Such changes represent a burned-out phase of the dermatomyositis skin rash and can be extremely difficult to treat.
Brief answer:
Medicines that applied directly to the skin in creams and ointments can help partially. However, most all patients require some form of systemic or internal medicine in the form of pills or shots to decrease the inflammation and/or suppress the autoimmune responses that are thought to cause dermatomyositis skin disease. The same internal treatments that are used to treat the muscle weakness of dermatomyositis are also used to treat dermatomyositis skin disease. Dermatomyositis skin disease is usually more difficult to treat than other more common autoimmune skin diseases such as lupus erythematosus (e.g., discoid lupus erythematosus [DLE], subacute cutaneous lupus erythematosus [SCLE)]).
More complete answer:
The brief answer to this question would be "not as effectively as doctors would like." For example, the skin rash of dermatomyositis is on average considerably more difficult to treat than the typical skin changes that occur in patients with lupus erythematosus. For example, approximately 75% of patients with discoid lupus erythematosus skin lesions (DLE) or subacute cutaneous lupus erythematosus skin lesions (SCLE) respond to one or a combination of the oral (pill-form) antimalarial drugs such as hydroxychloroquine (Plaquenil), chloroquine (Aralen) or quinacrine. However, a much lower percentage of patients having dermatomyositis skin rashes respond to these agents.
The same internal treatments that are required to treat the muscle weakness of dermatomyositis can result in improvement or clearing of dermatomyositis skin disease. At times these stronger forms of treatment that can produce a number of potentially severe side effects are also required to suppress the dermatomyositis skin rash when this is the only way the disease is showing itself. However, when only skin disease is present, most physicians prefer to treat the skin disease by applying medicines in creams and ointments directly to the affected areas of skin. Even when both skin disease and muscle disease is present, creams and ointment-based medications can be applied to the skin rash in order to minimize the doses of internal medications that are required to control the entire disease.
Topical forms of treatment
Before internal treatments are considered, most physicians begin with treatments that are applied directly to the skin. This form of treatment is referred to as "local therapy" or "topical therapy." Examples include sunscreens and corticosteroid-containing creams and ointments that can work when rubbed directly into the dermatomyositis skin rash.
Sunscreens. In most dermatomyositis patients, exposure to sunlight or artificial forms of ultraviolet light will make their skin problems worse. Thus, they should take precaution to prevent being directly exposed to sunlight (include a link to the Sun Tips part of home page). In addition, when patients are at risk of being exposed to sunlight, they should apply an effective broad spectrum sunscreen agent to all areas of exposed skin, including those area that are affected by the dermatomyositis skin. The particular sunscreen product should effectively block both the sunburning UVB ultraviolet rays as well as the longer wave length UVA rays. In addition, it should have a sun protection factor (SPF) of 30. A list of some such products that are currently available on the market without a prescription can be found elsewhere (include a link to a sunscreen recommendation page).
Topical corticosteroid. Topical corticosteroids are creams and ointments that contain a class of chemicals that is commonly referred to as "cortisone" by the public. These topical corticosteroid commercial products can differ greatly in their ability to suppress difficult skin problems such as the rash of dermatomyositis.
Vehicles: Different ways to get corticosteroid chemicals into the skin where they can do some good. Creams and ointments consist of a "vehicle" that can "carry" the active ingredient such as a corticosteroid chemical beneath the surface of the skin. If the chemical cannot get passed the protective barrier layer on the outer surface of the skin (known as the stratum corneum) it cannot do its job of decreasing inflammation or suppressing an autoimmune response.
White creams are favored by most patients since they disappear into the skin when rubbed on. However, clear ointments that tend to have a greasy, Vaseline-like feel are generally more effective at carrying the active ingredient into the skin. Therefore, the same concentration of an active ingredient in an ointment vehicle tends to give better relief than when delivered in a cream vehicle. The stronger, prescription forms of topical corticosteroids are usually required to make much of a difference in the appearance of the dermatomyositis skin rash and the severe itching that can accompanies it.
Differences in topical corticosteroid strength. One percent hydrosorticone (class 7) is the strongest topical corticosteroid chemical that is currently available over the counter in the United States without a prescription. This is a relatively weak form of topical corticosteroid that is usually of little help to dermatomyositis patients.
The stronger prescription-strength topical corticosteroid creams and ointments (class 1-2) can cause side effects in the skin because of their high potency. These include thinning of the skin and the formation of visible blood vessels (telangiectasia). Therefore, it is advisable to use them in the same areas of skin only sparingly and intermittently. Thus, for the very strong potency corticosteroid creams and ointments, patients are advised to use them daily or twice daily to the affected areas of skin for two weeks and then let those areas of skin rest without treatment for two weeks. This cycle can then be repeated for prolonged periods of time without great risk of side effects developing in the skin. However, daily use in the same areas of skin for long periods of time can cause the side effect problems mentioned above.
Different forms of topical corticosteroid are needed for different parts of the body. Certain parts of the body such as the scalp are especially challenging to treat with topical corticosteroids. Such areas require topical corticosteroids dissolved in other vehicles such as solutions, sprays, or gels. A potent corticosteroid solution massaged into the still-moist scalp after shampooing can provide the greatest benefit from this form of therapy. The foam and spray forms of topical corticosteroids can also be used in the scalp and other hairy areas of the body at other times during the day.
An ointment form of topical corticosteroid is generally going to be more effective when treating thickened, scaly, heaped-up lesions. Topical corticosteroid preparations should not be applied directly to open areas of skin such as ulcers since they can retard healing.
Topical corticosteroid side effects. One should avoid using the strongest topical corticosteroid preparations in certain areas of the body that are especially sensitive to these drugs. These include the face, neck, groin, under the breasts, between the buttocks. These areas of skin are naturally thin and subject to becoming even more thin when strong topical corticosteroids are applied. Visible blood vessels can also develop in such areas. These parts of the body should be treated with medium or low strength topical corticosteroid preparations (include a link to top. corticosteroid potency table).
The skin of the face is especially sensitive to stronger topical corticosteroid side effects such as thinning and rosacea. Mid potency or low potency preparations should be used on the face in the two week on-off cycles described above when prolonged therapy is anticipated. Care should also be taken when applying the stronger topical corticosteroids around the eyes for prolonged periods of time. In this setting, there have been reports of complications such as glaucoma (increased pressure inside the eyeball).
Rosacea (also called acne rosacea) is a very common red skin condition that affects the face of certain individuals. People who flush and blush a lot when embarrassed are especially prone to developing rosacea. The common form of rosacea can be readily treated with a combination of topical and oral antibiotics that are safe to take over prolonged periods of time. The use of stronger topical corticosteroid can also predispose one to developing a form of rosacea ("steroid-induced rosacea). Such drugs can also cause another rosacea-like skin problem, perioral dermatitis, to appear on the face.
Some patients can become allergic to chemicals used in the vehicles of topical corticosteroid preparations. This has been especially true of the chemical preservatives in cream vehicles. In addition, on very rare occasions, patients have even become allergic to the corticosteroid chemicals in topical creams and ointments. A clue to these forms of allergy would be if the skin rash becomes worse (redder and more itchy) rather than better after applying a cream or ointment. If you ever notice this, it is important to report this to your doctor.
Intralesional corticosteroid therapy. Corticosteroid chemicals can be injected into skin lesions that are not responsive to topical corticosteroid therapy with a needle and syringe. However, this is somewhat painful and needs to be repeated on a regular basis for maximal benefit. Thus, this is often not a practical form of long-term treatment of dermatomyositis skin lesions since they are often so numerous and affect large areas of the body.
Occlusive corticosteroid therapy. Another way to make medicines applied to the surface of the skin work better is to cover the treated are with a waterproff barrier. One such form of treatment is the use of tape that has been produced with a corticosteroid chemical trapped inside it (e.g., Cordran tape). Such medicated tape is cut to the shape of the lesion, placed on the skin rash, and left in place for 8-12 hours at a time. There is a very strong steroid effect on the underlying skin. Therefore, care should be taken not to over use this form of treatment because of the potential of higher producing steroid side effects.
Topical corticosteroid preparations become much more potent and effective when covered by plastic gloves, a plastic shower cap, or plain clear plastic food wrap . By trapping moisture in the skin with these measures, the steroid chemical tends to penetrate the barrier layer of the skin with much better. Thus, this form of treatment can help benefit when standard topical therapy does not. However, there is a greater complication of steroid side effects in the skin with such occlusive therapy.
Internal forms of treatment.
Unfortunately, only a small portion of dermatomyositis patients will have a good to excellent response to topical corticosteroid therapy alone. Because of the lack of availability of other proven forms of topical medicine that help in the rash of dermatomyositis, such patients require some type of internal (systemic) therapy. Most physicians would use one of the aminoquinoline antimalarial drugs as the first form of oral therapy for the skin rash of dermatomyositis.
Antimalarials. These drugs, such as hydroxychloroquine (Plaquenil) (at beginning indicate this format means brand name), tend to work quite slowly and should be given an 8-12 week trial period before attempting to determine whether additional treatment is necessary. Side effects of the antimalarial drugs in the daily doses that are currently used include a rare risk of eye complications. The retina (nerve layer ) of the eye can be affected. However, if one has regular eye examinations while on this class of drugs, the risk of losing vision is virtually non-existent. In some countries such as Great Britain, routine eye testing when on these drugs is not done. In addition, patients in the USA having other diseases such as rheumatoid arthritis that are treated with hydroxychloroquine are not required to have routine eye exams.
When treatment with a single antimalarial drug such as hydroxychloroquine or chloroquine is not able to suppress the skin rash of dermatomyositis, doctors often use combinations of antimalarial drugs (e.g., hydroxychloroquine + quinacrine or chloroquine + quinacrine). Like hydroxychloroquine, chloroquine also carries a very small risk of eye complications but quinacrine does carry such as risk. However, quinacrine can cause a yellow discoloration of the skin that is especially prominent on thick skin areas such as the palms and soles. This discoloration goes away once the drug is discontinued and is only of cosmetic significance.
Other anti-inflammatory drugs. Several other classes of medication can be used when treatment with antimalarial drugs is not successful at suppressing the dermatomyositis rash. Dapsone is give in pill form. Gold salts can be give as pills (Auranofin) or injections (Solganal). Like the antimalarials, these drugs have the advantage of not suppress the good parts of one's immune system. High doses of human gamma-globulin can also be of value in calming the skin and muscle inflammation in difficult cases of dermatomyositis. This form of treatment requires administration by needle into a vein (intravenous).
Immunosuppressive drugs. When all else has failed for the rash of dermatomyositis, internal (systemic) corticosteroids can be used. This class of drug can be given by mouth in pill form on a daily basis (prednisone). In especially sick patients, it can also be given on a monthly basis by intravenous injection (Solu-Medrol). Treatment with internal corticosteroid medications such as this is the most effective initial treatment for patients having dermatomyositis muscle weakness.
There are a number of complications that predictably will occur in all patients when high doses of systemic corticosteroids are given for prolonged periods of time. These include suppression of the immune system, mood disturbance (hyperactivity, depression), sleep disturbance, appetite stimulation, weight gain, rounding of the facial features (development of a moon-like face), tendency to develop diabetes mellitus and hypertension (high blood pressure), and weakening of bones (especially in the spine). The risk of developing all of these side effects are related to the amount of the drug being taken each day. Therefore, doctors are always trying to find a way to use as low a dose of these drugs as possible in order to minimize these side effects.
Like other drugs in this class, internal corticosteroid work in part by suppressing the abnormal part of the body’s immune system (i.e., the autoimmune responses). Unfortunately, they also suppress the good, protective parts of the immune system. Thus, patients treated with high doses of internal corticosteroid or the other immunosuppressive drugs mentioned below have a small risk of developing severe infections such as pneumonia that would be normally prevented by a healthy immune system. In addition, such patients have a small risk of developing certain forms of cancer such as lymphoma that are normally held in check by the immune system in healthy individuals.
In cases of especially difficult dermatomyositis skin disease, physicians can consider using other immunosuppressive drugs such as methotrexate, azathioprine (Imuran), cyclophosphamide (Cytoxan), chlorambucil (brand name), cyclosporine (Neoral), or mycophenolate (Cell-Cept). While there is a chance for complications of using immunosuppressive drugs, they can be quite helpful in patients having especially difficult forms of dermatomyositis skin disease and muscle disease. The doctor who treats you with such immunosuppressive drugs should be very experienced with the ins and outs of their usage. For example, blood tests should be done frequently while taking these powerful drugs.
Experimental forms of treatment. Several forms of topical and internal experimental therapy are currently being examined. Those include topical therapy with Tacrolimus. This is a potent immunosuppressive drug that is currently being used in pill form to prevent immunological rejection of kidney, heart, and liver transplants. A ointment form of this drug that can be directly applied to the skin has been developed. This drug has shown great promise in other inflammatory skin diseases such as atopic dermatitis (a form of eczema). There is reason to believe that it could also be of value in autoimmune skin diseases such as the rash of dermatomyositis. Studies in skin diseases such as atopic dermatitis have shown that this drug is quite safe. Not enough of the active ingredient is absorbed through the skin into the blood stream to affect the immune response in general.
Thalidomide, an medication that is taken by mouth that is very effective in treating difficult lupus skin disease patients. It is also possible that thalidomide could work in dermatomyositis skin disease. However, a great deal of caution must be taken to prevent pregnancy while on this drug as it can cause major birth defects in human including malformed arms and legs. In addition to sleepiness and constipation, thalidomide can also cause nerve damage in the arms and legs.
Brief answer:
There are several types of medicines that can help. Local measures include the liberal use of bland skin moisturizing creams and ointments such as original Eucerine (there is a saying in dermatology, "dry skin is itchy skin"). Also certain anti-itch medications such as products that contain pramoxine can be effective when applied directly to the skin. Topical corticosteroid creams and ointments can also help with itcihing.
Pill form internal medications can also combat itching. Examples include antihistamines such as (hydroxyzine [Atarax, Vistaril], cetirizine [Zyrtec], loratidine (Claratin), and fexofenadine [Allegra]). Tricyclic antidepressants such as doxepin used in low doses can also be helpful, especially when given at bedtime.
More complete answer:
The itching that characteristically accompanies dermatomyositis skin problems can at times be maddening. Special measures are often required for this problem in patients having dermatomyositis.
Skin dryness is often a problem in dermatomyositis skin disease patients and dry skin is always itchy skin. Skin dryness is a greater problem in people who are older. In addition, the low humidity of winter can also make dry, itchy skin worse. Therefore, effective moisturizers should be used at all times by patients with an itchy dermatomyositis skin rash. The liberal use of bland skin moisturizing creams and ointments such as original Eucerine or Aquaphor tend to work best. However, there are a number of other such good products. (insert link to dry skin information or include that here from prior Sjogren syndrome publications) While moisturizing lotions such as Vaseline Intensive Care and Lubriderm are quite popular, all lotions contain alcohol which can be drying.
There are various topical medications that can help itching. One class includes the topical corticosteroids as previously discussed. Topical corticosteroids decrease the itching by decreasing the inflammation caused by dermatomyositis in the skin. Other non-steroid topical anti-itch agents that can help include pramoxine. Specific products containing pramasone include Prax Lotion, Caladryl Clear Lotion. Both products as well as others are available without a prescription. These medications function as topical anesthetics that unfortunately have a relatively brief effect.
Alternatively, low percentages of chemicals such as phenol (0.5%) and menthol (0.25%-0.5%) can be added to moisturizing lotions for added anti-itch benefit. However, the relief provided tends to be relatively brief. One must take care in using some over-the-counter anti-itch preparations as they often contain sensitizing chemicals that can produce allergic reactions in the skin (e.g., antihistamines such as benzocaine). One should be suspicious of this if the itching and the rash are getting worse rather than better after rubbing something on.
Another approach would be the use of topical doxepin cream (Zonalon topical cream). However, this agent, too, can trigger allergic reactions in the skin in some susceptible individuals. In addition, over use can cause sleepiness.
Topical antibiotics such as polymyxin-bacitracin (Polysporin) or mupirocin (Bactroban) can help control infection in open or crusted areas of the skin. However, some topical antibiotics neomycin which is present in Neosporin can also cause skin allergy.
For oral treatment for itching, one can initially try the conventional antihistamines such as hydroxyzine (Atarax, Vistaril) or doxepin (Adapin, Sinequan). Since both tend to produce sleepiness, they are best used at bedtime. The antihistamines that do not produce drowsiness to a significant extent can be used regularly during the day. Examples include loratadine (Claritin), fexofenadine (Allegra), and cetirizine ([Zyrtec).
Recent studies have indicated that naltrexone (ReVia) might be useful in very severe cases of itching caused by medical conditions. This is an oral medication that blocks the action of opioid-type chemicals in the brain. There is hope that this drug could also help patients with dermatomyositis.
Classical dermatomyositis patients will occasionally experience symptoms of other autoimmune rheumatic diseases such as systemic lupus erythematosus and scleroderma. This has been referred to as mixed connective tissue disease or overlap connective tissue disease. This overlap pattern of illness is generally associated with a good prognosis. That is to say, such patients often do better with treatment than patients who have only the features of dermatomyositis. As previously mentioned, adults with classical dermatomyositis patients are at risk for developing an internal cancers of various types. Such patients are also at risk for developing a severe form of pneumonia. It is not certain that clinically-amyopathic dermatomyositis have an equal risk of these later two complications.
Dermatomyositis can first appear during pregnancy and some features of classical DM such as muscle weakness can be made worse by pregnancy. In several patients with clinically-amyopathic dermatomyositis, the skin rash has been observed to completely disappear during the second or third trimester of pregnancy. Changes in treatment could not explain this improvement. The dermatomyositis rash returned in each of these patients about 6-12 weeks following delivery of healthy babies.
In general, the development of babies is not affected by dermatomyositis. In addition, some of the internal medications used to treat DM patients such as systemic corticosteroid are quite safe during pregnancy. However, other internal drugs such as methotrexate, cyclophosphamide, and chlorambucil can cause problems with a developing baby.