The information below will be presented in the format of answers to questions that individuals with dermatomyositis skin problems and their family members frequently ask their doctors (dermatologists [skin specialists], rheumatologists [arthritis specialists], neurologists (nervous system specialists), internists, pediatricians, or family medicine physicians). Many of the terms that we will use to answer these questions might not initially be familiar to you. However, you can find explanations and definitions of most of the confusing terms in language that that you will be able to understand in the accompanying glossary (double click on the blue word "glossary" to go directly to this ist of definitions). The questions to be answered are listed in the box below to provide the reader an overview of the subject matter that will be discussed. Each of the following questions will be answered in two ways - a brief answer followed by a more complete, detailed answer.

1. What is dermatomyositis?

Brief answer:

Classical dermatomyositis is an autoimmune disease that commonly causes a characteristic skin rash and muscle weakness (See glossary for definition of unfamiliar terms such as "autoimmune.") On rare occasions, other vital internal organs such as the lungs, heart, bowels, and eyes can also be damaged. Involvement of some internal organs such as the bowels and eyes is seen more commonly in children with dermatomyositis compared to adults with this disease. In addition, certain internal complications such as calcium deposits in damaged tissue (i.e., calcification) are seen more commonly in childhood-onset dermatomyositis. However, adults with classical dermatomyositis have a relatively greater risk of developing internal cancers in associaton with their dermatomyositis.

The rash of dermatomyositis has a unique appearance and distribution over the body. The muscle weakness is most prominent in the shoulders, hips, neck and stomach, but muscles all over the body can be affected and become weakened. For example, the muscles used for swallowing food can be affected by dermatomyositis resulting in a choking sensation when one attempts to swallow solid foods or liquids. Some patients initially develop the skin rash but can go for 20 years or longer without experiencing muscle weakness (i.e., clinically-amyopathic dermatomyositis).

More complete answer:

Dermatomyositis is an autoimmune disease that causes damage to the skin (i.e., "dermato" - Latin for "skin") and certain types of muscles (i.e., "myo" - Latin referring to "muscles"). There are two forms of dermatomyositis - the classical form and the clinically-amyopathic form ("a" - meaning "no or without", "myopathic" meaning "muscle disease".

Classical dermatomyositis consists of a characteristic skin rash and muscle weakness most often noticed initially in the shoulders and hips. In clinically-amyopathic dermatomyositis, the skin rash can be present for long periods of time (six months or longer) without the appearance of muscle weakness (some patients having dermatomyositis skin disease been observed to go 20 years or longer without developing muscle weakness).

The rash of dermatomyositis initially appears as a red-purple (violet-colored), itchy skin change. This rash is most prominent on the upper eyelids (heliotrope erythema), the cheeks of the face, the outside or hairy surface of the arms, the backs of the hands and knuckles of the fingers, elbows, outer hips and thighs (holster sign), knees, and inside bony part of the 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 to sunlight, over the back of the neck and shoulders (shawl sign), 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 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 fleshy borders of the fingernails (nailfold areas). Tiny but visible blood vessels appearing as fine red lines can be seen perpendicular to the nail fold areas. In addition, the cuticle of the fingernails often gets very ragged, overgrown, 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 (calcinosis). These can feel like pebbles or small stones under the skin's surface.

The initial way that patients notice that their muscles are being affected by dermatomyositis is one or a combination of pain, tenderness, and weakness. This is usually first noted in the shoulder and hip muscles. Activities such as raising one's arms to comb one's hair or to remove dishes from a kitchen cabinet become difficult to perform. Patients also frequently find it difficult to arise out of a chair from a sitting position without using one's hands on the chair arms to help push oneself up. Also, it can be difficult to arise when one stoops over to pick something up off the floor. Weakness in the legs can often be more severe than in the arms resulting in great difficulty in climbing stairs, getting into or out of a car, walking long distances, or running.

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 several months before an individual notices muscle weakness or other symptoms of dermatomyositis muscle disease. Patients can be 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 the act of swallowing solid foods or liquids and can produce a choking sensation. The muscles used in chewing and speaking can also become weak. 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.

Other bodily organs besides the skin and muscles can on occasion be injured by dermatomyositis. Those include the lung, heart, bowels, and eyes. A form of pneumonia not caused by infection can be especially severe in dermatomyositis patients and can even cause death. This form of pneumonia is classified as a form of "interstitial lung disease" or "interstitial pneumonitis." It has been estimated that 5-40% of patients with classical dermatomyositis will eventually develop some degree of interstitial lung disease. In addition to occurring in patients with classical dermatomyositis, this form of pneumonia has also been recognized to occur in clinically-amyopathic dermatomyositis patients although the relative frequency has not yet been determined. Certain patterns of internal organ injury that can occur in dermatomyositis are seen more frequently in childhood-onset dermatomyositis than adult-onset dermatomyositis (e.g., bowel injury, eye injury).

Classical dermatomyositis patients are also at risk for certain internal medical complications. One such problem is the development of different types of cancers inside the body. When women with dermatomyositis develop internal cancer, they 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 having a extensive skin rash are at the greatest risk for developing cancer. In this setting, approximately 1 in 4 classical dermatomyositis patients develop some type of internal cancer within 2 years before to 2-4 years after the onset of their dermatomyositis. It is not yet known if patients with clinically-amyopathic dermatomyositis have an equally high risk of developing internal cancers. However, such individuals should have the same type of cancer evaluation as those with classical dermatomyositis until more information is available concerning this risk.

It is thought that this increased risk of developing internal cancers disappears within 2-4 years after a patient is diagnosed as having classic dermatomyositis. During that period, dermatomyositis patients should be carefully observed for the development of any signs of internal cancer. In some patients, when the internal cancers have been successfully treated, the dermatomyositis symptoms markedly improve or disappear. 2. How is the diagnosis of dermatomyositis made?

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 (the skin biopsy can narrow the possibilities down to dermatomyositis versus lupus skin disease -- the clinical appearance of fully-blossomed dermatomyositis skin disease is quite different than that of lupus.). A number of other conditions can cause muscle weakness. Therefore, diagnosing dermatomyositis as the cause of muscle weakness can be much more challenging. One or a combination of the following tests can be required to confirm the diagnosis of dermatomyositis as the cause of muscle weakness: blood tests (muscle enzymes, autoantibodies), tests of muscle electrical activity (electromyogram), special x-ray imaging of muscles (MRI, muscle spectroscopy), sound wave testing of muscles (ultrasonography), and muscle biopsy.

More complete answer:

When the skin rash of dermatomyositis has fully developed, it can be diagnosed by its appearance alone if a physician has had sufficient experience in this area (dermatologists generally have the highest level of such training and experience). 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 there are specific autoantibodies (myositis-specific antibodies) that can be identified in the blood of patients with classical dermatomyositis (e.g., Mi-2, Jo-1). The preliminary results of a recent study have suggested that a different set of autoantibodies (155 kd, Se) can also be seen in the blood of patients with 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 into the skin causing mild discomfort. The characteristic electromyographic changes of dermatomyositis if present can be diagnostic of dermatomyositis muscle damage. Muscle biopsy is often performed by a surgeon 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 dermatomyositis. A muscle biopsy is done by surgically removing a small piece of muscle tissue under local anesthesia (patient is awake) or general anesthesia (patient is asleep). Experimental studies are being carried to determine the accuracy of muscle biopsy obtained by inserting a hollow needle through the skin into the muscle.

Imaging studies such as magnetic resonance imaging (MRI), muscle spectroscopy and muscle ultrasonography are being used more and more, but still remain primarily research tools. 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 inflamed (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.

3. What causes dermatomyositis?

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 likely to be required. When exposed to certain virus infections, sunlight, or chemicals, individuals who are genetically predisposed tend to develop autoimmune reactions that damage the skin and muscle tissue (and sometimes other organs) such as the lungs 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. Autoantibodies such as antinuclear antibodies and myositis-specific autoantibodies bind to proteins normally present inside one's own normal skin and 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, ultraviolet rays from sunlight, and/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, the Epstein-Barr virus, and others. However, infection alone does not appear to be the only cause of the faulty immune responses that are thought to cause dermatomyositis. One's particular set of genetic traits also appears to be important. If one inherits the combination of genes (probably 10-20) that predisposes to dermatomyositis from one's parents, then one is susceptible to developing dermatomyositis when exposed to triggering virus infections, sun rays, or chemicals. Research studies are currently being carried out to identify which particular genes are responsible for causing the abnormal immune responses that are thought to be responsible for dermatomyositis. Some scientists believe that certain viruses that can infect muscle cells can change 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. 4. What is known about clinically-amyopathic dermatomyositis? Brief answer: Unfortunately, very little is known since no formal research studies have been carried out to date on this form of dermatomyositis. Our current understanding of this type of dermatomyositis results mostly from a series of medical case reports only. This form of dermatomyositis can occur in both adults and children. Muscle weakness develops very slowly in some patients only after a n umber of years. However some clinically-amyopathic dermatomyositis patients have been observed to go 20 years and longer without any evidence of muscle weakness. A small number of clinically-amyopathic dermatomyositis patients have developed severe lung disease even to the point of death. Whether this form of dermatomyositis has a true increased risk of internal cancer development is not yet known. More complete answer: This is a rare form of dermatomyositis in which the very same highly-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. Even though they are not experiencing muscle weakness, some patients with clinically-amyopathic dermatomyositis have evidence of very subtle muscle abnormalities when they undergo certain forms of muscle testing (e.g., electromyography, muscle biopsy, muscle spectroscopy). The long term significance of such muscle testing abnormalities is not known at this time, however some such patients have been observed to go for many years without developing muscle weakness. This subgroup of patients has been referred to as "hypomyopathic dermatomyositis" for the purposes of clinical research studies. Clinically-amyopathic dermatomyositis is a poorly understood subgroup of dermatomyositis patients. It is not known whether such patients truly have a significantly 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. 5. What are the risk factors for developing dermatomyositis? Brief answer: Both classical dermatomyositis and amyopathic dermatomyositis can develop at any age and in both sexes. The specific genes that make one susceptible to the development of dermatomyositis have yet to be identified. Some viral infections are thought to be trigger factors for dermatomyositis. In addition, exposure to sunlight can aggravate the DM skin rash. More complete answer Dermatomyositis can occur in both sexes and at any age. There are two age peaks for developing classical dermatomyositis - during childhood and in middle aged adults. Women are somewhat more commonly affected than men with classical dermatomyositis. This gender imbalance is even more pronounced in clinically-amyopathic dermatomyositis. Both the classical and the clinically-amyopathic dermatomyositis forms of dermatomyositis can during childhood and the teen years. 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 dermatomyositis 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 (both skin rash and muscle weakness) have a higher complication rate from damage to internal blood vessels. This can result in catastrophic complications such as the death of a segment of the bowel inside the abdomen. In addition, damage to retinal layer blood vessels 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 classical dermatomyositis are not at increased risk for developing internal cancers. It has recently been observed that there is a childhood-onset form of clinically-amyopathic dermatomyositis. That is, children can have only the skin changes of dermatomyositis for abnormally prolonged periods of time (> 6 months) 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. The specific genes involved have not yet been identified. Thus, genetic testing to predict the occurrence of dermatomyositis or its complications is not yet possible. Although genetics is thought to be important in the cause of dermatomyositis, it is very unusual for more than one close family member to be affected by this disease. 6. How is the skin first affected in dermatomyositis and how does it change over time? Brief answer The very earliest symptom is usually an itchy, red-purple (violet-colored [violaceous]) change in skin color that develops in a characteristic pattern over the body. Over time, the rash can deepen in color, become scaly, and occasionally develops blisters that can break down to produce erosions or ulcers. Without treatment, the skin rash tends to be very persistent and long-lasting. More complete answer The very earliest symptom is usually a red-purple skin change that is quite itchy. The areas of the skin that are affected tend to be the upper eyelids, the scalp, the nape of the neck, backs of the shoulders, the cheeks of the face, the open collar V-area of the neck and upper chest, the outer (hairy) aspects of the arms and forearms, elbows, backs of the hands, knuckles, and nail-fold areas, outer hip areas, knees and occasionally the inner aspect of the ankles. If the skin rash is very severe blisters and ulcers can develop. Involvement of the scalp can result in hair loss. However the hair can grow back completely when the skin rash is controlled by treatment. Longstanding skin disease can result in a combination of thin skin overlying areas displaying dark and light coloration as well as with visible blood vessels (telangiectasia). This combination of skin changes is called "poikiloderma' and is seen most commonly over the V-area of the neck and upper chest, back, flanks and buttocks. In many patients, once the rash appears in these areas, it tends to slowly extend to involve larger areas of the skin, assume a darker hue, and becomes even more itchy. After a while, a fine scale can develop over the involved areas of skin. In addition, small scaly bumps can appear around the base of the hairs as they project out of the skin on the outer aspects of the arms, forearms, hips and thighs (follicular hyperkeratosis). Skin involvement in some severely-affected patients can progress to a point where most of the skin over the body is affected with a miserably itchy, red-purple rash. The itching can be so severe as to cause sleep deprivation and occupational as well as psycho-social disability. Also, as mentioned earlier, blisters can form in severely-inflamed dermatomyositis skin lesions. When these blisters break down, they can be very slow to heal and sometimes can produce superficial open area (erosions) or deeper open areas (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 (cutaneous calcinosis). This tends to occurs most frequently around the elbows, forearms, and hands but can develop almost anywhere on the body. As previously mentioned, the tendency to develop calcinosis is magnified in juvenile-onset dermatomyositis). Some patients with clinically-amyopathic dermatomyositis can have one or more abnormal muscle test although they continue to have no muscle weakness for long period of time. 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 dermatomyositis and clinically-amyopathic dermatomyositis are thought to be identical in appearance to the eye and under the microscope. It might be useful at this point to review some of the unconventional disease terms relating to dermatomyositis patients that do not have muscle weakness that are being used in this article. The term "amyopathic dermatomyositis" as used here refers to patients that have diagnostic combinations of the characteristic skin changes of dermatomyositis for abnormally long periods of time (6 months or longer) without developing muscle weakness. We use the term "hypomyopathic dermatomyositis" here to refer to patients who meet the just-mentioned definition for amyopathic dermatomyositis but who are found to have asymptomatic evidence of mild muscle abnormalities upon some form of muscle testing. We use the term "clinically-amyopathic dermatomyositis" as an umbrella designation to refer to both amyopathic DM and hypomyopathic DM patients, since their only medical symptoms relate to their skin problems (i.e., clinically-amyopathic dermatomyositis = amyopathic dermatomyositis patients + hypomyopathic dermatomyositis patients). 7. Do the skin problems of dermatomyositis ever go away? Brief answer Yes. But in most patients skin problems return after treatment is stopped. However, a small percentage of patients will experience a "one-time" type of illness where their disease goes away with treatment and does not return even after treatment is stopped. More complete answer The answer to this question is "yes." Some patients develop dermatomyositis skin disease and then have their disease symptoms 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. Children who develop dermatomyositis have a somewhat better chance than adults of having all their disease problems suppressed by treatment and never having further disease activity. In some patients with classical dermatomyositis, skin disease activity returns without the return of muscle weakness. Thus, some patients that initially have the dermatomyositis rash and muscle weakness, relapse with only the skin rash when their disease activity returns following withdrawal of treatment. In this setting, the skin rash is the patient's main day-by-day medical problem. The skin rash in this situation can be very difficult suppress with treatment. Such patients often require some form of systemic treatment to suppress the overactivity of the immune system. Such immunosuppressive treatments can be given by mouth (orally) 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 obscure terms dermatologists use to indicate 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. 8. How is dermatomyositis skin disease treated? Brief answer Medicines that are applied directly to the skin in the form of creams, ointments, gels, solutions, sprays, or foams can help partially. However, most dermatomyositis skin rash patients require some form of systemic (internal) medicine in the form of pills or shots to decrease the inflammation and suppress the autoimmune responses that are thought to cause dermatomyositis skin disease. The same types of internal treatments that are used to treat the muscle weakness of dermatomyositis when present are also required to treat dermatomyositis skin disease. Dermatomyositis skin disease tends to be 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 could be "not as effectively as doctors an patients 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. 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 (formerly available by the brand name, Atabrine, however this form of the drug is no longer available in the USA). (When a specific drug is mentioned in this discussion, it will be listed by its generic [i.e., chemical] name first followed in parenthesis by its brand-name. Brand name drugs always begin with a capital letter while generic names begin with lower case letters.) However, a lower percentage of patients having dermatomyositis skin rashes respond to these agents. It should be noted that drugs in the antimalarial class such as hydroxychloroquine (Plaquenil), chloroquine (Aralen) or quinacrine can themselves occasionally cause side effects in the muscles (e.g., a vacuolar myopathy) that can be confused for activity of dermatomyositis muscle disease activity.) 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 carry the risk of 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 skin disease is the only symptom of dermatomyositis that is present, most physicians prefer to initiate treatment of the skin disease by applying medicines in various topical vehicles (creams, ointments, gels, solutions, sprays, or foams) directly to the affected areas of skin. Even when both skin disease and muscle disease is present, topical cream- 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. Sun avoidance and sun protection. In most dermatomyositis patients, exposure to sunlight or artificial forms of ultraviolet light (UV) will make their skin problems worse. It is the UV rays in sunlight that present the greatest danger to dermatomyositis patients (UV rays are also present in certain types of artificial light sources such as tanning lamps, germicidal lamps and welding apparatus). Natural sunlight at the earth's surface contains two types of UV rays - ultraviolet-B (UVB) and ultraviolet-A (UVA). UVB rays, which are the sunburning rays, are thought to present the greatest danger for aggravating dermatomyositis skin disease. However, the longer wavelength rays of ultraviolet light (UVA) can also cause problems. Harmful UV rays reflect off water and light colored surfaces (e.g. concrete, sand, and snow) so that the amount of UVB radiation exposure to the skin may be doubled. UV rays also reach below the surface of water; in fact, three feet of water blocks only 20% of UV rays. Sunscreen should be applied daily to dry skin about 15 to 30 minutes before going outdoors even on cloudy days when up to 80% of UV rays can still reach the earth's surface. In addition, repeated mini exposures to UV radiation may account for 80% of total exposure over a lifetime. Thus, daily use of sunscreens throughout the year is advisable. Patients should avoid artificial tanning devices, wear lightweight tightly woven clothing and broad brimmed hats, and use broad spectrum, water resistant sunscreens. A practical guide for sun protection is available line for patients on the Internet at http://tray.dermatology.uiowa.edu/SafeSun/SafeSun-Index.html. Patients should be advised to avoid direct sun exposure, particularly during 10 AM and 4 PM especially during the summer months when the UV component of sunlight is least filtered through the atmosphere. A useful rule of thumb is that if one's shadow is longer than one is tall, i.e. when the altitude (which is the angle above the horizon) of the sun is less than 45 degrees, there is relatively less danger from UV radiation exposure. Applying the above principle, one may figure out the time of the day to avoid direct sun exposure anywhere in the world. It is also a good idea for patients to check the UV Index each day and dress accordingly. The UV Index is a prediction of the sun's UV radiation on any given day at noon in a particular geographic region. Local daily UV Index is available via the local newspaper, television, radio, or the Internet (www.cpc.ncep.noaa.gov/products/stratosphere/uv_index/uv_current.html). Hats and protective clothing. A hat brim of four inches or greater is recommended and patients should make certain that the top and brim of a straw hat have sun-proof liners in place. Small-brim hats (less than 2.5 cm) provide a sun protection factor (SPF) of 1.5 for the nose and minimal shield for the chin. Medium-brim hats (2.5-7.5 cm) offer a SPF of 2 for the cheek and a SPF of 3 for the nose. Broad-brim hats (>7.5 cm) provide a SPF of 3 for the cheek, an SPF 7 for the nose, an SPF of 5 for the neck, and SPF of 2 for the chin. There are lightweight plastic hats that are commercially available and designed specifically to provide a physical block to UV radiation (e.g., Sun Helmets [www.sunhelmets.com]). Typical summer shirt fabrics only offer SPF of 6.5. Weave tightness is the most important factor in sun protection of fabrics followed by the fabric type. Cotton and polyester/cotton blends offer comparable photoprotection. When stretched, the Lycra fabric is significantly less effective than when it is lax. Darker color fabrics provide greater photoprotection than do lighter color fabrics. It is also important to note that fabrics are significantly less photo-protective when wet. Several clothing lines offering maximized UV protection (e.g. SPF 30) are currently being marketed and are easily accessed through the Internet (e.g., Solumbra Ultra Sun protective Clothing [sunprecautions.com]; Frogwear Sun Protective Clothing [1-800-328-4440]; MasqueRays [sunproof.com]; Sun Protective Clothing [sunprotectiveclothing.com]; SunGrubbies [sungrubbies.com]). Such sun-protective specialty clothing is also marketed for fishermen and those going on safaris. UV blocking films should be applied to home and automobile windows and acrylic diffusion shields should be placed over fluorescent light tubes/bulbs to block the small amount of UV irradiation that can leak from such sources (UVA>UVB). A number of companies offer UV light blocking films or plastic shields that can be applied to home and automobile windows. This is important because whereas window and car glass material may offer some shield against UVB, they are transparent for UVA. More information on these products can be obtained through the Internet (e.g. Solis Films [southwall.com]; North Solar Screen [northsolarscreen.com]; Llumar UV shield window film [llumar.com]). An explanatory letter from a physician is generally sufficient for individuals to get permission from law enforcement agencies to apply darkly colored UV protective film to automobile windows. Sunscreens/Sunblocks. The sun protection factor (SPF) is defined as the dose of UV radiation required to produce 1 minimal erythema dose (MED) on unprotected skin (one can think of the "minimal erythema dose" as a "minimal sunburning dose"). Currently, the SPF value listed on a sunscreen product applies solely to UVB protection (thus, the degree of UVA protection cannot be determined from this information). Some sunscreen products provide more UVA protection than others. Patients should select broad-spectrum sunscreens that contain agents that block UVB and UVA with an SPF of 30 or greater. SPF 15 blocks 92% of damaging rays and SPF 30 blocks 96% ultraviolet rays. It has been found that much lower amounts of sunscreens are actually used in real life situations compared to the amounts employed under lab conditions for determining the SPF rating of a specific sunscreen product. In fact, patients and consumers normally apply only _ to _ the amount of the sunscreen product that was used by the company in their initial tests to determine the SPF value. Therefore, when a photosensitive patient uses a SPF 30 sunscreen in real life, they in reality often get a SPF of about 15 or less, which is the minimum necessary for adequate protection for dermatomyositis patients. The average adult requires a minimum of one ounce of sunscreen for adequate total body coverage. Sunscreen products containing UVA physical blockers such as titanium dioxide and zinc oxide or UVA chemical blockers like Parsol 1789 (also known as avobenzone) and Mexoryl-Sx provide the broadest degree of UVA protection. Sunscreen that offers UVA coverage is important because 50% of the exposure to UVA occurs in the shade, i.e. in the absence of direct sunlight. Popular use of sunscreens containing titanium dioxide or zinc oxide may be limited by their opaque cosmetic appearance. Parsol 1789 provides excellent protection from UVA but undergoes significant degradation under UV exposure that it may lose 50 to 90% of its UVA protective effectiveness after just one hour under the sun. Sunscreen products that are most resistant to being washed off by sweating or bathing should be selected. "Water-resistant" sunscreens protect skin for 40 minutes of water exposure whereas "waterproof" sunscreens protect for 80 minutes. Gels work well for oily skin or when sweating. Lotions help dry skin and sprays work best on the body. Stick or lip balm-type sunscreens that are formulated for use on the lips can also be applied around the eyes to avoid the eye irritation that often occurs when other products are applied to this sensitive area. Stick-type sunscreens can also be used to gain maximum sun protection to the ears. Foundation makeup products without sunscreen may not offer more than a SPF of 4 via its pigment content. Corrective camouflage cosmetics such as Dermablend [dermablend.com] and Covermark [covermark.com] offer the dual benefit of being highly effective physical sunscreens as well as elegant cosmetic masking agents for patients suffering psychologically from chronic, treatment-resistant disfiguring skin disease. Avoidance of sun-sensitizing drugs. Certain medications can magnify the effects of UV rays on the skin and thereby aggravate sun-sensitive skin problems such a dermatomyositis. Examples include piroxicam and other anti-inflammatory pain relievers, sulfonamides (an antibiotic), tetracycline (an antibiotic), and hydrochlorothiazide (a diuretic medication used to treat high blood pressure). Drugs used to treat diabetes mellitus and psychological/psychiatric disorders can also cause sun-sensitivity. If you feel that you might be experiencing a worsening of your dermatomyositis skin problems after starting a new medication, discuss this with your doctor. Strategies for preventing or minimizing skin dryness One of the most troubling symptoms for many patients with dermatomyositis skin disease is severe itching (pruritus). The treatment for itching in this setting with medications that are applied directly to the skin and are given by mouth will be discussed below. However, dryness of the skin for any reason always worsens the symptom of itching. In addition, the skin changes of dermatomyositis themselves can cause or aggravate skin dryness. Therefore, the discussion immediately below is provided to help dermatomyositis patients better treat and prevent skin dryness. What does dry skin look like? Dry skin is a very common skin problem and is often worse during the winter when environmental humidity is low (i.e., "winter itch"). It can occur at all ages and in people with or without other skin problems like dermatomyositis. Everyone is familiar to some degree with the appearance of dry skin. The normally fine lines in the skin become more visible, the skin feels rough and appears dull and flaky. In more advanced cases, fish net-like cracks resembling the fine fracture lines of cracked porcelain can occur. Dry skin occurs most commonly on the arms and legs, but can also affect the trunk of the body. Dermatologists often call dry skin "xerosis" or "asteatosis". Problems associated with dry skin. Dry skin very commonly produces itching, which can be severe and interfere with sleep and other daily activities. Repeated rubbing and scratching can produce areas of thickened, rough skin (lichenification). Dry, thickened skin can crack, especially in areas subject to chronic trauma (e.g., hands and feet), causing painful cracks in the skin (fissures). Dry skin and scratching may result in a dermatitis when the skin becomes red (inflamed) in addition to dry and scaly. Round, scaly, itchy, red patches scattered over the legs, arms and trunk (nummular eczema) may also appear. The appearance of yellow crusts or pus in these areas indicates that a bacterial infection is developing. This would require specific antibiotic therapy from your dermatologist or family physician. If your skin is very dry, or if you have an associated red dermatitis, it is a good idea to seek the advice of your dermatologist or family physician. Severe dry skin is a feature of certain genetic diseases such as atopic dermatitis and ichthyosis (fish scale-like skin). In addition, people with hormone imbalances such as underactivity of the thyroid gland can also experience severe skin dryness (there are other changes in the body that accompany dry skin in this setting that helps doctors recognize this problem). On occasion red, dry skin rashes can be confused with other skin problems such as a ringworm infection or allergic contact dermatitis (i.e., a poison ivy-like skin rash), which would need different forms of treatment. What causes dry skin? Healthy skin can be pictured as a multi-layer cake covered by a single sheet of clear plastic food wrap to keep it fresh. The plastic food wrap prevents the frosting and underlying layers of the cake from drying out by preventing loss (evaporation) of the water from the cake into the air. It is the moisture in the cake that gives it its freshness. The outermost layer of the skin, called the stratum corneum, acts like the plastic food wrap and is about the same thickness (this is the dead skin layer that peels off after a sunburn). The stratum corneum consists of toughened dead skin cells embedded in a mixture of natural oils (lipids) that are made by underlying living skin cells. These natural skin oils keep the water inside our body from escaping into the air and also keep irritating substances and germs from entering the body. Both the skin oils and the dead skin cells hold a certain amount of water in the stratum corneum and it is this stratum corneum water that helps keep the skin soft, pliable and smooth. Dry skin results when there is not enough water in the stratum corneum for it to function properly. One way this can happen is when protective oils in the stratum corneum are lost and the water that is normally present in the skin is allowed to escape. Too much soapy water, exposure to harsh chemicals, the normal aging process and certain types of skin diseases are some of the causes of decreased amounts of protective skin oils. As the stratum corneum dries out it shrinks and, as it shrinks, small cracks can occur. This exposes the underlying living cells to irritating substances and germs in the environment. Treatment of dry skin An important aspect of treatment is to identify and tackle any factors that may be contributing to the dry skin. It is natural to think that applying water alone to dry skin would help control the problem. However, water alone (especially hot water) can actually worsen the problem of dry skin by removing the normal, protective skin oils. Hot, soapy water depletes the natural skin oils to the greatest degree. (Anyone who has tried to wash a greasy skillet in cold soapy water knows how effective heat is in softening up oils and fats so that they can be washed away.) However, water followed by the application of oil such as a moisturizer (also known as an emollient or lubricant) is of great benefit for dry skin. The oil in the moisturizer helps trap and seal water in the skin and makes it softer, smoother and less likely to become dry, cracked and itchy. A mainstay of dry skin treatment is attention to proper bathing techniques and the liberal use of effective moisturizers. You should take a short bath or shower (no more than 10 minutes) only once in a 24 hour period. For adults, showers are generally better than baths. While longer baths or showers, especially in hot water, can be quite relaxing, they will also increase the loss of natural oils from the skin and worsen skin dryness. The bath or shower should be in lukewarm rather than hot water. Soap should be used minimally and only when and where needed (for example, under the arms, the groin and genitals, the feet, and the face). Milder, less drying soaps include Dove, Neutrogena Dry Skin Formula (unscented), Aveeno Cleansing Bar for Dry Skin, Purpose, Basis, and Oil of Olay Sensitive Skin Soap. Cetaphil is a soap-free liquid cleanser that works as a gentle and effective soap substitute for some people. It is especially helpful for cleaning the face and hands. After bathing or showering, quickly and gently pat the skin partially dry with a towel (do not rub!). Within three minutes of getting out of the water apply a moisturizer (see discussion of moisturizers below) to seal the water in the skin before it can evaporate. Bath oil, if selected as a moisturizer, can be directly rubbed into the skin after showering or bathing, but should not be added to bath water since this can make the tub dangerously slippery resulting in falls and broken bones. Moisturizers should be reapplied liberally during the day and evening when possible especially to those areas especially prone to dryness (hands, arms, legs) and when itchy. One should be careful about using non-prescription, over-the-counter anti-inflammatory and itch-suppressing creams or lotions. Many of these products contain chemicals that can irritate or cause allergic reactions in dry, dermatitic skin. A good general rule is that if anything that you apply to your skin causes more burning and itching than you started with, you should stop using it until you can talk with your doctor about it. Over-the-counter anti-itch products containing pramoxine (e.g., Prax, Pramasone) or menthol and camphor (e.g., Sarna) are generally safe to use. However, these products are not treating the cause of skin dryness, they are only temporarily treating the itching that accompanies skin dryness. Any way that you can increase the humidity level in the air of your home and workplace would be advisable. If not already present, you should consider adding a humidifier to the central heating system of your home. Portable humidifiers are relatively inexpensive and can be used in the bedroom at night. Use of skin moisturizers (emollients) for long term control and prevention of dry skin. Dry skin is usually a long term problem that recurs often, especially in winter. When you notice your skin beginning to get dry, resume your moisturizing routine and avoid the use of harsh soaps. If the itchy, dry, skin rash returns, use both the moisturizers and the prescription steroid cream or ointment. Types of moisturizers There are basically two types of moisturizers - facial moisturizers and body moisturizers. Most facial moisturizers on the market relate largely to makeup and cosmetic concerns. These products are surrounded by considerable mystique and are often outrageously expensive. These products are different than body moisturizers in that they are very carefully designed to avoid causing allergic reactions (hypoallergenic) and flares of acne on the face (non-comedogenic). Facial moisturizers used for cosmetic purposes will not be discussed further here. While dryness of the skin of the face is usually not a major problem for people during the winter, the use of certain medications such as anti-acne treatments can produce considerable facial dryness in the winter or summer. There are several excellent facial moisturizers that also contain effective sunscreen chemicals that can be used in this setting (Neutrogena Moisture SPF 15 Untinted, Cetaphil Daily Facial Moisturizer SPF 15). There are four basic classes of body moisturizers Ü ointments, oils, creams and lotions (listed in decreasing order of moisturizing power). It should be noted that all of the moisturizer products mentioned in this article are available without prescription. Ointment moisturizers have the greatest ability to trap moisture in the skin, but they have the greasy consistency and feel of Vaseline Petroleum Jelly. People often shy away from using them because of the greasy feel, but this can be minimized by applying a small amount and rubbing it into the skin well (when the skin takes on a sheen). Examples of effective ointment moisturizers include Aquaphor and plain Vaseline Petroleum Jelly. In addition to brand name moisturizers, some common household products, such as Crisco vegetable shortening, can be used as very inexpensive body moisturizers. Again, the key to using an ointment is to apply small amounts and rub it in well. Oil moisturizers are less greasy but still effective. Examples of oils that can be applied directly to the skin include baby oil, mineral oil, vegetable oil, and bath oil. Bath oils used in the bath water make the tub too slippery and should not be used. It is preferable to apply bath oils after getting out of the tub or shower, just as you would other moisturizers, directly to damp skin immediately after a light toweling off to partially dry the skin. Cream moisturizers are usually white and disappear when rubbed into the skin without leaving a greasy feel. As a result they tend to be more popular than ointments. Examples of effective cream moisturizers include Original Eucerin Cream, Aquaphilic, Cetaphil Moisturizing Cream, Vaseline Cream, Eutra/Elta, Nutraderm, Moisturel, Nivea, Nutraplus, Complex-15, Carmol, Pen-Kera, and Neutrogena Hand Cream-Norwegian Formula (especially helpful for dry, chapped hands and cuticles). Lotion moisturizers are suspensions of oily chemicals in alcohol and water. Lotion moisturizers are generally the least greasy and the most pleasant to use and therefore are quite popular. However, because of their alcohol content, they can be somewhat drying when used repeatedly compared to ointments and creams. The bottom line is that if the moisturizer you choose does not feel at least a bit greasy you may not be getting as strong a moisturizer as you might need. Examples of popular lotion moisturizers include Vaseline Intensive Care, Nutraderm Keri, Lubriderm, Curel, and Complex 15, Nivea, U-Lactin, Neutrogena, Nutraplus, Eutra Lite, Moisturel, Complex-15, Lac-Hydrin 5. There are many other cream and lotion moisturizers on the market that have not been listed here because of space constraints. We have chosen to list some of the more popular products and those more often recommended by dermatologists. Some moisturizers contain chemicals that can cause skin irritation or allergic reactions in some people (e.g., fragrances, preservatives, alpha hydroxyacids, urea, sunscreens). Thus, if one brand of moisturizer gives you problems, try another in the same class that has a different set of ingredients. Within a given moisturizer class, the choice of which one to use is a matter of personal preference. Generally speaking, the greasier the feel of the moisturizer, the more it traps and holds water in the skin. To be fully effective, it is necessary to use a moisturizer three or more times daily, in the same way that chapped hands in the winter need many treatments. As mentioned before, the most important time during the day to use a body moisturizer is right after a short lukewarm bath or shower. Some material above was excerpted from the following Internet sites that we consider to be reliable: http://www.dermnet.org.nz/index.html http://www.kaiserpermanente.org/toyourhealth/library/skintypes.html http://my.webmd.com/content/asset/adam_symptoms_dry_skin Topical forms of treatment for DM skin disease 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, ointments, gels, solutions, sprays, or foams that can work when rubbed directly into the dermatomyositis skin rash. Topical corticosteroids. Topical corticosteroids products are most often creams and ointments that contain a class of chemicals (corticosteroids) that are commonly referred to as "cortisone" by the public. Very weak forms of topical corticosteroids (e.g., 1% hydrocortisone cream or ointment) are available currently in the USA without a prescription. However, dermatomyositis skin rashes usually require much stronger forms of topical corticosteroids and all such products require a prescription. 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 past the protective barrier layer on the outer surface of the skin (known as the stratum corneum) it cannot do its job of decreasing the skin inflammation of dermatomyositis. 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 chemical 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. As mentioned above, 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 often-severe itching that can accompany it. Differences in topical corticosteroid strength. One percent hydrocortisone cream (e.g., Cortaid) (class 7) is the strongest topical corticosteroid chemical that is currently available over the counter in the United States without a prescription. This is weak form of topical corticosteroid that is usually of little help to dermatomyositis patients. Prolonged use of 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 side effects 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 any topical treatment for two weeks. This cycle can then be repeated for prolonged periods of time without great risk of side effects developing in the areas of treatment. 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. Because the hair interferes with the application of creams and ointments, the scalp requires a corticosteroid chemical dissolved in other vehicles such as solutions, gels, sprays, or foams. A potent corticosteroid solution massaged into the still-moist scalp after shampooing can provide the greatest benefit from this form of therapy. The gel, 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. The spray form with its long thin nozzle applicator is especially convenient for use during the day. An ointment form topical corticosteroid is generally going to be more effective than any other type when treating thickened, scaly, heaped-up lesions. Topical corticosteroid preparations should not be applied directly to open areas of skin such as erosions or ulcers without the direction of your physician since they can retard healing. Topical corticosteroid side effects. One should avoid using the medium and strong 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 thinner when strong topical corticosteroids are applied. Visible blood vessels can also develop in such areas. These parts of the body should be treated with low strength topical corticosteroid preparations. 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 strong topical corticosteroids around the eyes for prolonged periods of time. In this setting, there have been reports of eye complications such as glaucoma (increased pressure inside the eyeball). Rosacea (also called acne rosacea) is a very common inflammatory (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 ("corticosteroid-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 themselves 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. This is done with a tiny needle (30 gauge) and a syringe. However, this can be a bit painful, especially if larger needles are used. In addition, it often needs to be repeated on a regular basis for maximal benefit (every 4-6 weeks). 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 water-proof barrier. One such form of treatment is the use of a special tape that has been manufactured with a corticosteroid chemical trapped inside it (e.g., Cordran tape). Such medicated tape is cut to the shape of the skin area to be treated, placed onto this area, and left in place for 8-12 hours at a time. There is a very strong corticosteroid effect on the skin underlying such tape. Therefore, care should be taken not to over use this form of treatment because of the higher chance of producing steroid side effects in the skin. Topical corticosteroid preparations become much more potent and effective when covered by plastic gloves, a plastic shower cap, or 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 much more efficiently. Thus, this form of "occlusive" topical treatment can help when standard topical therapy does not. However, there is a greater risk for complications of steroid side effects in the skin with such occlusive therapy. Interesting new forms of topical immunosuppressive medication such as tacrolimus (Protopic) are currently being examined as possible new types of local treatment for DM skin disease. Internal forms of treatment for dermatomyositis skin disease. 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 experienced dermatologists 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 (USA brand name-Plaquenil), tend to work quite slowly and should be given at least 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 very rare risk of eye complications (antimalarial retinopathy). The retina (nerve layer) of the eye can be affected. However, if one has regular eye examinations every 6-12 months by an eye specialist (ophthalmologist) 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 while on these drugs is not felt to be cost-effective and is not done. 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 and because of this, it is recommended that these two drugs not be used at the same time. Fortunately, quinacrine does not 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. Since blood cell problems have on rare occasions occurred with the use of quinacrine, most physicians order a routine complete blood count (CBC) intermittently. 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 given in pill form. 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 (IVIG) can also be of value in calming both the skin and muscle inflammation in difficult cases of dermatomyositis. This form of treatment is extremely expensive and requires administration by needle into a vein (intravenous). Another oral medication, thalidomide (Thalomid), is currently being studied informally for possible benefit in the skin problems experienced by dermatomyositis patients. 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 especially around the face (moon-like face), neck and body; tendency to develop diabetes mellitus (i.e.,, "sugar diabetes") and hypertension (high blood pressure); and weakening of bones (especially those in the spine). The risk of developing all of these side effects is related to the total dose (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 or blood infections (sepsis) that would be normally prevented by a healthy immune system. In addition, such patients have a small risk of developing certain malignancies especially in the immune system itself (e.g., 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 (Leukeran), cyclosporine (Neoral), or mycophenolate mofetil (Cell-Cept). Methotrexate is the drug that most physicians have used as a corticosteroid-sparing drug in severe dermatomyositis skin disease. 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 sufficiently 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 was initially approved for use in pill form (Prograf) to prevent rejection of kidney, heart, and liver transplants by the immune system. An ointment form of this drug (Protopic) that can be directly applied to the skin has been developed and was recently approved for use in atopic dermatitis (a form of eczema) patients. 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, even for use in children as young as 3 years of age. Not enough of the active ingredient is absorbed through the skin into the blood stream to affect the immune response in general. Thalidomide (Thalomid), a medication that is taken by mouth that is very effective in treating patients with difficult forms of skin lupus, a disease that shares features with dermatomyositis skin disease. 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 occasionally cause nerve damage in the arms and legs, and thus electrical coduction within nerves of the arms and legs must be carefully monitored. 9. What can I do for the itching that accompanies my dermatomyositis skin disease? Brief answer There are several approaches that can help to control itching (pruritus) in dermatomyositis patients. Local measures include the liberal use of skin moisturizing ointments, creams, lolions and oils (as described in depth under question #8 above). Over-the-counter anti-itch products containing pramoxine (e.g., Prax, Pramasone) or menthol and camphor (e.g., Sarna) are generally safe to use. However, these products are not treating the cause of skin dryness, they are only temporarily treating the itching that accompanies skin dryness. 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 (10-50 mg at bedtime) can also be helpful (video taping has documented that patients with itchy skin diseases like dermatomyositis scratch themselves while sleeping, often not being aware of this the next morning). More complete answer The itching that characteristically accompanies dermatomyositis skin problems can at times be so severe as to interfere significantly with one's social life. In some, it can be downright 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 should receive special attention, as described in depth under question #8 above. 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 in the skin caused by dermatomyositis. Other non-steroid topical anti-itch agents that can help control itching symptomatically include pramoxine. Examples of products containing pramoxine include Prax Lotion and Caladryl Clear Lotion. Both products as well as others are available without a prescription. Alternatively, low percentages of chemicals such as phenol, menthol, and camphor can be added to moisturizing lotions for added anti-itch benefit (e.g., Sarna Lotion). However, the relief provided tends to be relatively brief and overuse of such products can produce skin irritation (e.g., contact dermatitis). These medications function as topical anesthetics that unfortunately have a relatively brief effect. Some of these products contain chemicals that can irritate or cause allergic reactions in dry irritable skin. A good general rule is that if anything that you apply to your skin causes more burning and itching than you started with, you should stop using it until you can talk with your doctor about it. Another approach would be the use of a prescription topical itch cream such as doxepin cream (Zonalon Cream). However, this agent, too, can trigger allergic reactions in the skin in some susceptible individuals. In addition, over usage can cause sleepiness due to absorption of the doxepin chemical through the skin into the body (special caution should be used with topical forms out doxepin such a Zonalon when a patient is already taking doxepin by mouth). Itching causes scratching and scratching often produces small breaks (excoriations) in the protective surface layer of the skin. Such open areas of skin are prone to becoming infected with the bacteria (germs) that normally live on the skin. Non-prescription 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 that contain the antibiotic neomycin (e.g., Neosporin, Triple Antibiotic Ointment) can cause skin allergy in predisposed individuals (allergic contact dermatitis). For oral treatment for itching, one can initially try the conventional prescription antihistamines. Diphenhydramine (Benadryl), although available without a prescription, generally produces too much sleepiness to be of value in managing the itching of dermatomyositis skin rashes. Better effects can be achieved with the prescription antihistamines, hydroxyzine (Atarax, Vistaril) or doxepin (Adapin, Sinequan). Since both hydroxyzine and doxepin tend to produce sleepiness, they are best used at bedtime. Doxepin can be especially helpful in this situation since it has a long half-life in the blood, providing relief throughout the evening. The antihistamines that do not produce drowsiness to a significant extent can be used regularly during the day. Current 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 might also help dermatomyositis patients with severe itching. 10. What other diseases or complications can be associated with dermatomyositis? Brief answer The following complications can occur in dermatomyositis patients: cancer, arthritis, pneumonia, other autoimmune diseases (systemic lupus erythematosus, scleroderma, autoimmune thyroiditis, diabetes mellitus, infections and other medication side effects. All new problems such as fever and chills, chest pain, shortness of breath, abdominal pain, pain in only one arm or leg, weakness in only one arm or leg, and vision disturbances should be discussed with one's doctor. More complete answer Cancer can complicate dermatomyositis as has been previously discussed. Patients with classical dermatomyositis can also suffer from arthralgia (joint aches) or arthritis (tender, painful swollen joints). Classical dermatomyositis patients will occasionally experience arthritis and other symptoms of different autoimmune rheumatic diseases such as systemic lupus erythematosus and scleroderma. This mixture of problems has been referred to as "mixed connective tissue disease" or "overlap connective tissue disease." This overlapping type 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. Occasionally, other gene-based autoimmune diseases such as autoimmune thyroid disease and juvenile-onset diabetes occur in patients with dermatomyositis. As previously mentioned, adults with classical dermatomyositis have an increased risk of developing internal cancers of various types and are also at risk for developing a severe form of pneumonia (interstitial pneumonitis). It is not certain that clinically-amyopathic dermatomyositis have an equally increased risk of these later two complications. Strong internal medical treatments that are necessary to control the autoimmune features of dermatomyositis. Drugs like prednisone, methotrexate, and azathioprine can make one's body susceptible to serious infections. In addition, each of these drugs can produce their own specific side effects. It is important that you understand the possible side effects of each drug you are given. Your physician and pharmacist should provide you with this information - if not, you should ask for it. In addition, much reliable information including drug side effects is readily available to everyone through the Internet. If one does not have personal access to the Internet this can often be obtained through a public library. The symptoms of dermatomyositis usually affect both sides of the body equally. Thus, if one experiences a problem only or mostly on one side of the body, this can be a sign of complication. Severe pain in one arm or leg can signify an evolving bacterial infection. Such problems should be brought to the attention of your physician. The development of any of the following new problems should be discussed with one's doctor: fever and chills, chest pain, abdominal pain, pain in only one arm or leg, weakness in only one arm or leg, and vision disturbances. 11. What effect does pregnancy have on dermatomyositis? Brief answer 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 dermatomyositis 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. 12. Is my family at risk for getting dermatomyositis? Brief answer It is possible but extremely unlikely that your close relatives might develop dermatomyositis or related diseases. More complete answer While this is possible, it is extremely unlikely. Since dermatomyositis is thought to result from a combination of genes rather than just a single gene, it is quite unlikely that one's children or other direct blood relatives would inherit the same combination of genes that you did as a dermatomyositis patient. However, some of the genes that predispose to dermatomyositis (e.g., certain HLA tissue typing genes) are the same ones that predispose an individual to other forms of autoimmune disease (this could help explain why some dermatomyositis patients are at risk for developing overlapping features with other rheumatic diseases such as lupus and scleroderma,). Thus, in addition to there being a tiny risk of your other family members developing dermatomyositis, it is also possible that they might be at a small risk of developing other autoimmune connective tissue diseases such as lupus, scleroderma, and autoimmune thyroid disease. If there is any concern by any of your family members in this regard, they should consult their individual physicians. 13. I have heard that children can also get dermatomyositis. Do children have the same problems with dermatomyositis as adults? Brief answer Yes, children can develop dermatomyositis. Juvenile (or childhood)-onset dermatomyositis has both similarities and differences from adult-onset dermatomyositis. More complete answer: Unfortunately, what you have heard is true - children of all ages can develop a form of classical dermatomyositis (skin and muscle disease). However, their disease tends to be somewhat different than that experienced for the first time during adulthood. First of all, children with dermatomyositis (i.e., juvenile dermatomyositis, childhood dermatomyositis) do not appear to be at risk for developing internal cancers in association with their dermatomyositis as are adults. In addition, dermatomyositis in children often results in more blood vessel damage than does that experienced by adult-onset dermatomyositis patients. This can result in a higher risk for the development of calcium deposits in areas of blood vessel damage and resulting tissue damage (i.e., calcinosis). Calcinosis can be a serious complication since it can produce ongoing tissue problems long after the active inflammation from dermatomyositis has subsided. The increased risk for blood vessel damage seen in juvenile dermatomyositis patients can produce severe damage in various internal organs including the gastrointestinal tract (stomach, intestines and colon) and the retinal layer of the eye. It has recently been recognized that amyopathic dermatomyositis can also develop in children. It is not yet known whether children with amyopathic dermatomyositis are at risk for developing non-muscle internal dermatomyositis complications such as interstitial lung disease as are adults with amyopathic dermatomyositis.

14. Are there any lifestyle changes that I can make to help control the activity of my dermatomyositis? For example, should I change my diet, take any herbals or other health food supplements, avoid stress?

Brief answer

Not much other than protecting yourself from unnecessary sun exposure, minimizing life stress as much as possible, and leading as healthy a lifestyle as possible.

More complete answer:

As previously discussed, dermatomyositis patients should protect themselves from unnecessary sun exposure as much as possible.

Both emotional and physical stress can worsen closely-related autoimmune diseases such as systemic lupus erythematosus. And, clearly physical exertion can aggravate muscle disease in patients with classical dermatomyositis. However, less is known about the impact of emotional stress on the skin and muscle changes of dermatomyositis. It is the experience of some physicians, including the author, that emotional stress can worsen some disease features of both classical and clinically-amyopathic dermatomyositis.

Cigarette smoking has been show to interfere with the beneficial effects of antimalarial drugs on the skin problems experienced by lupus patients. Whether the same is true for the skin changes of dermatomyositis is not certain. However, because of the many similarities between the skin rashes of lupus and dermatomyositis, it would be prudent for dermatomyositis patients to avoid cigarette smoking while taking antimalarials and at other times as well for general health considerations.

Physicians routinely recommend good nutrition and physical therapy to help patients recover from the muscle weakness caused by classical dermatomyositis. However, there is virtually no scientific evidence that dietary factors play a significant role in causing or treating either the muscular or skin problems experienced by dermatomyositis patients. Nor is there such scientific evidence that herbals or other forms of health food supplements play a role in the treatment of any form of dermatomyositis.

However, physicians do often offered dietary advice to patient suffering from closely-related autoimmune disorders such as systemic lupus erythematosus. It is really not known how much such dietary restrictions might benefit a patient with dermatomyositis skin disease. However, since a number of these recommendations are the same as those made for good health in general, I will review them here in outline form.

The following information was excerpted from an article entitled "The prudent gourmet: How the answer to 'What's for dinner?' affects your health" by Dr. Scott Glickstein, M.D. that was published in the April/May, 2001 issue of Lupus Awareness (a newsletter published by the Lupus Foundation of America):

Lupus patients are advised to consider adhering to the "prudent diet" recommended by the American Heart Association, American Diabetes Association and the Surgeon General's Office. The percentage of calories one derives from each of the three major food groups should be: carbohydrates, 50-60%; protein, 20-30%; and fats, 20-30%. The lower the concentration of fat in the diet, the better, as for as heart disease and atherosclerosis (hardening of the arteries) is concerned.

Fiber supplements should be considered Calcium supplements should be considered. This is in especially important for anyone taking oral corticosteroids (e.g., prednisone) for prolonged periods of time as treatment for their dermatomyositis. Daily calcium intake should equal approximately 1500 mg of elemental calcium. This should be accompanied by taking a full daily requirement of vitamin D (400 U.).

Avoiding foods that contain large amounts of alfalfa seeds and sprouts as well as herbal supplements that contain alfalfa. Ingestion of large amounts of a chemical present in alfalfa seeds (L-canavanine) has been related to triggering a lupus-like illness in humans and monkeys. It should be emphasized that very large amounts of alcohol for ingestion occurred in this the settings. The small amount of alfalfa sprouts that are sometime used to garnish salads and sandwiches do not appear to cause problems for lupus patients. Fish oil supplements can be considered.

Ingestion of fish oil that contains omega-3 fatty acids has been shown to lessen certain types of inflammatory reactions. (Recall that dermatomyositis is caused by inflammatory reactions in skin and muscle tissue.). Dietary supplementation with omega-3 fatty acids has been shown to be beneficial in experimental animal models of lupus and lupus-like disease. However, the overall effect of omega-3 fatty acids on human systemic inflammatory diseases like lupus and perhaps dermatomyositis is thought to be small. Another possible benefit of omega-3 fatty acid dietary supplementation is a tendency to lessen the risk of atherosclerosis. However, adequate amounts of daily fish oil supplement can be quite expensive ($75/month).

Anti-oxidant supplements can be considered. Antioxidant dietary supplements have also been shown to be of benefit in experimental animal models of lupus or lupus-like disease. However, antioxidant dietary supplementation does not appear to have the same magnitude of benefit to human patients with systemic lupus erythematosus. There appears to have been no formal studies of antioxidant food supplementation in dermatomyositis to date.

Another potential benefit is that formal studies have indicated that antioxidant supplements may have some value in preventing or recovering from atherosclerosis (like lupus patients, dermatomyositis patients on long-term corticosteroid therapy are have an increased risk for atherosclerotic complications such a heart attack and stroke). Examples of generally available anti-oxidant supplements include: beta-carotene (a water soluble form of vitamin A), vitamin C, vitamin E; lycopene, and selenium. The best forms of antioxidant supplementation are thought to be through fresh foods that are enriched in these chemicals. For Vitamin A or Beta Carotene these include cantaloupes, mangos, carrots, sweet potatoes and red peppers; for Vitamin E - corn oil margarine, peanut butter, wheat germ and avocado; for Vitamin C: orange juice, potatoes, strawberries and kiwifruit. Lycopene is present in tomatoes and tomato products as well as other red fruits (watermelons, pink grapefruits, apricots and guavas). Alternatively, supplements of these antioxidant chemicals can be obtained without a prescription in any pharmacy.

Daily multivitamin supplement should be considered. A multivitamin tablet contains daily requirements for folate and Vitamin B complex which are thought to be important in lowering blood levels of a chemical called homocysteine. An elevated homocysteine level in the blood has recently been found to be a risk factor for atherosclerosis and its complications.

A lipid (fat)-lowering drug should be considered if one has elevated blood fat levels (cholesterol and triglyceride). This can be especially important for dermatomyositis patients taking drug treatments such as oral corticosteroids (e.g., prednisone [Deltasone, Orasone], prednisolone [Delta-Cortef Oral, Prelone Oral], or methylprednisolone [Medrol-Oral) that have a tendency to raise blood fat levels (the same is true if one is taking corticosteroids by needle injection on a regular, long-term basis). Your doctor should be following your blood fat levels if you are taking an oral or injectable corticosteroid drug for prolonged periods of time. Your doctor will advise you whether or not you might need a prescription form of a lipid lowering drug (e.g., Lipitor, Zocor, niacin). The problem in this setting with the drug class that includes Lipitor and Zocor is that they can produce side effects in the muscles that can be confused with the symptoms caused by dermatomyositis muscle inflammation.

15. Some of the information that you have discussed so far seems to be most relevant to white people (Caucasians) with dermatomyositis. Are there differences in how dermatomyositis skin disease affects individuals of other ethnic backgrounds?

A: Brief answer

The characteristic violet (heliotrope) skin color seen in white people with dermatomyositis is not as evident in individuals with more heavily pigmented skin.

More complete answer

The characteristic violet (heliotrope) skin color seen in white people with dermatomyositis is not as evident in individuals with more heavily pigmented skin. This is especially true when the skin disease of dermatomyositis first starts to develop. Thus, the diagnosis of dermatomyositis might be delayed somewhat in individuals having more heavily pigmented skin. This would be especially true for African-Americans. This fact could be a contributing factor to the observation that minority children in the US have a longer interval between the first juvenile dermatomyositis symptom and diagnosis/therapy than Caucasian children. However, socioeconomic differences resulting in varying access to health care are also thought to contribute to this difference. Otherwise dermatomyositis and dermatomyositis skin disease does not appear to very much with respect to race or ethnic background.

PubMed - the search engine for medical publications maintained by the National Library of Medicine www.pubmed.gov