Dermatomyositis is a progressive connective tissue disorder characterized by inflammatory and degenerative changes of the muscles and skin. Associated symptoms and physical findings may vary widely from case to case. Muscle abnormalities may begin with aches and weakness of the muscles of the trunk, upper arms, hips, and thighs (proximal muscles). Muscles may be stiff, sore, and tender and, eventually, show signs of degeneration (atrophy). Affected individuals may experience difficulty in performing certain functions, such as raising their arms and/or climbing stairs. In addition, affected individuals may experience speech and swallowing difficulties.
Skin abnormalities associated with dermatomyositis often include a distinctive reddish-purple rash (heliotrope rash) on the upper eyelids, across the cheeks and bridge of the nose in a "butterfly" distribution, the forehead, or additional skin regions; scaling and degenerative (atrophic) changes of affected skin on the extending surfaces of the knuckles, elbows, knees, and/or other regions (Gottron's sign); an abnormal accumulation of fluid (edema) in body tissues surrounding the eyes; and/or other features.
The symptoms of childhood dermatomyositis are similar to those associated with the adult form of the disorder. However, onset is usually more sudden. In addition, abnormal accumulations of calcium deposits (calcifications) in muscle and skin tissues as well as involvement of the digestive (gastrointestinal [GI]) tract are more common in the childhood form of dermatomyositis.
Although the exact cause of dermatomyositis is not known, it is thought to be an autoimmune disorder.
In those with dermatomyositis, the onset of symptoms may be gradual (insidious) or sudden (acute). The symptoms often wax and wane for no apparent reason.
The major symptom of the disorder is muscle weakness, most often affecting the trunk and muscles closest to the trunk (i.e., proximal muscles), such as the hips, thighs, shoulders, upper arms, and neck. The affected muscles may be stiff, sore, and/or tender and, eventually, may show signs of degeneration and muscle wasting (atrophy). Muscle weakness and degeneration progress and may lead to an awkward manner of walking (gait) and a gradual inability to perform certain tasks, such as lifting the arms, climbing steps, or dressing. Characteristic signs may include an inability to raise the head from the pillow when lying down or to rise unassisted from the floor. In some with the disorder, involvement of muscles of the neck, tongue, and/or throat may eventually result in difficulties swallowing (dysphagia) and/or articulating speech (dysphonia). In rare cases, weakening of muscles of the chest wall and diaphragm may cause respiratory difficulties, potentially leading to life-threatening complications without prompt, appropriate treatment. In addition, in some with chronic, long-term disease, certain joints may become fixed in a permanently bent (flexed) position (contractures).
Individuals with dermatomyositis also develop characteristic skin changes that, in some cases, may precede muscle weakness. Skin abnormalities often include a distinctive reddish-purple or lilac (i.e., heliotrope) rash that may be present on the upper eyelids (heliotrope eyelids); the forehead; the cheeks and bridge of the nose (“butterfly rash”); and/or other regions. In addition, affected skin on the extending surfaces of certain joints, such as the knuckles, elbows, knees, or other regions, may tend to become scaly, with central areas of tissue loss (atrophy) that lack or have increased pigmentation (Gottron’s sign). A “dusky” reddish (erythematous) rash may also develop on the upper arms, upper legs, and upper trunk. Although the skin rashes frequently fade completely, they may be followed by brownish discolorations (hyperpigmentation) of the skin, atrophy, scarring, and/or loss of color (depigmentation) of patches of skin (vitiligo). Dermatomyositis may also be associated with distinctive changes of the nails, such as bluish-red, scaling lesions around the base of the nails, reddish “shininess” of the nail folds, abnormal widening (dilation) of capillaries of the nailbeds, or other findings. Additional skin manifestations may include an abnormal accumulation of fluid in body tissues (edema) surrounding the eyes (periorbital area) and in other facial regions; an increased sensitivity to light (photosensitivity); and/or other findings. In some cases of long-term chronic disease, atrophic changes may result in binding of skin to underlying tissue structures. Some affected individuals, particularly children, may also have abnormal accumulations of calcium deposits (calcifications [calcinosis]) in affected muscles and tissues under the skin (subcutaneous tissues), potentially leading to contractures and localized muscle atrophy.
Some individuals with dermatomyositis may also develop generalized systems and findings, such as low-grade fever, a general feeling of ill health (malaise), pain in multiple joints (polyarthralgia), weight loss, enlargement of the liver and spleen (hepatosplenomegaly), and/or other abnormalities. In some cases, affected individuals, particularly those who have muscle inflammation (myositis) in association with other connective tissue disorders, experience Raynaud’s phenomenon. This is a condition in which sudden contraction of blood vessels supplying the fingers and toes (digits) causes a temporary interruption of blood flow and associated numbness, tingling, abnormal discoloration of the digits, and pain. Episodes are most commonly triggered by exposure to cold temperatures. (For more information, choose “Raynaud” as your search term in the Rare Disease Database).
Muscle weakness and impairment may progress to affect other areas of the body, including muscles of the GI tract, within the cardiovascular system (i.e., heart, blood vessels, and blood circulation), and/or the lungs. In such cases, associated symptoms and findings may include disease of the heart muscle (cardiomyopathy); impaired transfer of nerve impulses (conduction) that control the activity of heart muscle (heart block); irregular heart beats (arrhythmias); and/or inflammation of the lungs involving certain lung tissues (interstitial pneumonitis) resulting in breathing difficulties (dyspnea) and coughing.
In some individuals with dermatomyositis, there may be an association with an underlying cancer (malignancy). Reports indicate that the malignancy may precede, occur in association with, or develop subsequent to the onset of dermatomyositis. Malignancy-associated dermatomyositis appears to occur more frequently in individuals over age 40 years, particularly among those older than age 60 years. Although there is no characteristic cancer site or type, experts indicate that underlying malignancies appear to most commonly arise in the GI tract, lungs, breast, ovary, testis, or certain white blood cells (lymphocytes) or lymphoid tissue (e.g., certain lymphomas and leukemias, multiple myeloma). The relationship between dermatomyositis and malignancies is not fully understood.
The symptoms and physical findings associated with childhood dermatomyositis are similar to those observed in adult dermatomyositis. Onset is usually more sudden (acute) than in the adult form and often involves skin manifestations followed by muscle weakness. Calcification of muscles and tissues is more frequent and widespread in childhood dermatomyositis. Affected children also tend to have widespread inflammation of blood vessels (vasculitis), with more frequent involvement of the GI tract. In those with GI vasculitis, associated findings may include abdominal pain; difficult, infrequent, or incomplete passing of stools (constipation); or the passage of tarry, black stools (melena) or vomiting of blood due to the development of sores or eroded areas in the lining of the GI tract (bleeding peptic ulcers). Malignancy is rarely associated with the childhood form of dermatomyositis.
In rare cases known as dermatomyositis sine myositis, the skin abnormalities associated with dermatomyositis may occur without the associated muscle abnormalities.
The specific underlying cause or causes of dermatomyositis remain unknown. However, evidence suggests that genetic, immune, and environmental factors play some role. Underlying genetic and immune mechanisms may be suggested by various findings, including an increased frequency of certain genetically determined HLAs or “human leukocyte antigens” in individuals with the disorder. HLAs, which are proteins that play an important role in the body’s immune system, influence the outcome of transplantation and appear to affect an individual’s predisposition to certain diseases. Evidence suggests that certain HLAs have an increased frequency in children and adults with dermatomyositis. However, the specific implications of such findings are not fully understood.
Dermatomyositis is thought to belong to a group of disorders in which the body’s natural immune defenses inappropriately act against the body’s own tissues (autoimmune disorders). In dermatomyositis, an abnormal immune reaction appears to lead to obstructive inflammatory changes of blood vessels within muscle, connective tissues of the skin, and other tissues; patchy degeneration, wasting (atrophy), and regeneration of muscle fibers; thinning of the outermost skin layer (epidermis); and/or other associated findings.
Reports indicate that dermatomyositis may sometimes appear to occur subsequent to certain viral infections. Some researchers suggest that, in genetically predisposed individuals, the presence of foreign viral proteins (antigens) that are similar to certain of the body’s own proteins may trigger the production of antibodies that improperly “cross-react” or act against the body’s own cells (e.g., intramuscular blood vessels), leading to the inflammatory changes and muscle tissue damage seen in dermatomyositis. In addition, researchers indicate that the association of dermatomyositis with underlying malignancies in some adults may suggest an abnormal autoimmune response directed against a common antigen in muscle and the cancerous tumor. There have also been some reports in which dermatomyositis has appeared to occur following certain vaccinations or the use of particular medications. However, the implications of such findings remain unknown.
Dermatomyositis may occur at any time from infancy through approximately age 80, but most commonly it occurs between 40 to 60 years. In children, the symptoms usually appear between the ages of five to 15 years. Approximately three in 1,000,000 children are affected by juvenile dermatomyositis. Females are affected by Dermatomyositis twice as often as males.
Dermatomyositis may be diagnosed based upon a detailed patient history, thorough clinical examination, detection of characteristic physical findings, and certain specialized tests. Diagnostic findings include the presence of the characteristic skin rash; progressive weakness of proximal muscles; elevated levels of certain muscle enzymes (e.g., creatine kinase [CK], aldolase) in the liquid portion of the blood (serum) that may be suggestive of muscle inflammation; and abnormal findings on electromyography (EMG). EMG is a test that records electrical activity in skeletal (voluntary) muscles at rest and during muscle contraction. In some instances, physicians may also recommend magnetic resonance imaging (MRI), during which a magnetic field and radio waves create detailed cross-sectional images of certain tissues. In addition, muscle biopsies may sometimes be recommended to help detect certain changes that may help to confirm the diagnosis. Muscle biopsy involves the removal and microscopic examination of small samples of muscle tissue. (Experts indicate that muscles recently tested with EMG should be avoided since EMG procedures may cause inflammatory changes potentially leading to false positive results upon biopsy.)
Although the results of certain blood tests may provide helpful information suggestive of the diagnosis, such findings may be nonspecific, meaning that they may be associated with numerous autoimmune connective tissue disorders. For example, some affected individuals may have an elevated erythrocyte sedimentation rate (ESR). ESR measures the rate that red blood cells (erythrocytes) settle to the bottom of a test tube. Because erythrocytes tend to clump together and thus settle more quickly when inflammation is present, an elevated ESR functions as a nonspecific indicator of inflammation. In some affected individuals, testing may also reveal certain antinuclear antibodies (ANAs), which are “self antibodies” (autoantibodies) that react with antigens in the nuclei of cells. A number of specific autoantibodies have been identified that may be seen in some individuals with inflammatory muscle diseases, including dermatomyositis and polymyositis, that may be helpful in classifying the disease in certain cases (e.g., anti-Jo-1 antibodies, often seen in associated lung involvement).
In some cases, additional tests may also be recommended to help detect or characterize certain abnormalities that may be associated with the disorder. For example, x-ray imaging may reveal calcifications in certain soft tissues. In addition, stool samples may be taken to detect blood, a finding suggestive of gastrointestinal involvement.
For adults with dermatomyositis, the possibility of an underlying malignancy should be considered during basic clinical assessment. In addition to a basic, thorough physical examination, including breast, gynecologic, and/or rectal examination, screening may include routine blood testing, analysis of urine and stool, chest x-rays, mammograms in women, and/or other tests, as well as appropriate follow-up testing as required.
The treatment of dermatomyositis is directed toward the specific symptoms that are apparent in each individual. Such treatment may require the coordinated efforts of a team of medical professionals, such as pediatricians or internists; physicians who specialize in the diagnosis and treatment of connective tissue disorders (rheumatologists); specialists in the functioning of the immune system (immunologists); and/or other health care professionals.
Corticosteroids, particularly prednisone, are widely used in the treatment of dermatomyositis. Such medications, which are similar to the natural hormones produced by the outer region of the adrenal glands (adrenal cortex), are often used to reduce inflammation and associated swelling and also serve to suppress immune responses (immunosuppressive therapy). Blood levels of muscle enzyme (e.g., CK) activity are repeatedly measured to help monitor the effectiveness of such therapy. Reduction of these enzymes toward or reaching normal levels is noted in most affected adults within approximately six to 12 weeks after treatment is started, with subsequent improvement in muscle strength. When normal enzyme levels have been achieved, the dose of prednisone is usually slowly reduced. Dose levels are increased if muscle enzyme levels rise. Physicians may attempt to carefully, gradually withdraw such therapy periodically to determine whether the disease remains clinically active. In many cases, however, prolonged maintenance therapy with prednisone may be necessary. Yet reports indicate that some adults may appear to achieve a complete response. In such instances, therapy may be gradually withdrawn with careful ongoing monitoring.
Affected children may initially require high dose therapy with prednisone, which often results in a return of muscle enzyme levels toward normal levels within about one to two weeks. As with adults, decreased muscle inflammation and improved muscle strength typically follow. Dose levels may then be slowly reduced to the lowest possible dose sufficient to maintain normal enzyme levels and improve symptoms. Generally, according to experts, children with dermatomyositis may be able to discontinue prednisone after approximately two years, experiencing an apparent cessation of symptoms (i.e., remission).
High dose corticosteroid therapy may have adverse side effects, particularly after prolonged use, such as a decrease in bone density, causing bones to become brittle and weakened (osteoporosis); increasing, “superimposed” muscle weakness due to effects of the medication (i.e., corticosteroid myopathy); tissue swelling (edema); peptic ulcers; or other findings. Physicians may recommend certain measures to help prevent or minimize adverse effects, such as appropriate, alternative day therapy; administration of proper calcium and vitamin D supplementation; or the use of other medications (e.g., H2-receptor blockers). In some affected individuals, certain adverse effects may necessitate a decrease in dosage or discontinuation of therapy and substitution of another appropriate treatment.
Therapy with other immunosuppressive drugs, such as azathioprine, methotrexate, cyclophosphamide, chlorambucil, or cyclosporine, may be beneficial for some affected individuals who have an insufficient response to corticosteroid therapy alone, dose-limiting adverse effects, or frequent relapses. For example, preliminary investigations have shown that some affected individuals may benefit from combination therapy with azathioprine and corticosteroids, with the addition of immunosuppressant therapy often allowing the use of lower corticosteroid doses. In addition, reports indicate that some affected individuals have obtained benefit from methotrexate therapy for five years or more. Therapy with such immunosuppressive agents may have serious adverse effects, such as an increased susceptibility to infections and other effects. Thus, as with corticosteroid therapy, individuals who undergo treatment with immunosuppressive agents require ongoing monitoring to ensure appropriate response, to help minimize or manage possible side effects, and to make any necessary dosage adjustments or treatment substitutions. (For further information, please see the “Investigational Therapies” section below.)
In affected individuals with an associated malignancy, reports suggest that dermatomyositis often improves with removal of the underlying cancer*. Experts indicate that individuals with malignancy-associated dermatomyositis may also sometimes respond to therapy with corticosteroids such as prednisone. (*Depending upon the specific form, stage, and grade of the malignancy and other factors, recommended cancer treatment may include surgical removal of the malignancy; administration of certain anticancer drugs [chemotherapy]; radiation therapy; and/or other measures. During radiation therapy, radiation via x-rays or other sources of radioactivity is passed through selected regions of the body to destroy cancer cells and shrink tumors.)
In some cases, physical therapy may be recommended to help improve muscle strength and avoid the development of contractures. For individuals with calcifications, physicians may sometimes recommend surgical removal of calcium deposits. Treatment for other findings potentially associated with dermatomyositis (e.g., difficulty swallowing and/or breathing; speech problems; and/or abnormalities of the heart, lungs, and/or GI tract) is symptomatic and supportive. Affected individuals should be closely monitored by physicians so that proper preventive measures may be taken to help avoid serious complications potentially associated with this disorder.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
The National Institutes of Health (NIH) is seeking families in which an adult or child has been diagnosed within the previous four years with any of the diseases listed below. There also needs to be a twin or sibling of the same gender as the affected person who does not have an autoimmune disease. The twin or sibling should be no more than four years older or younger than the affected person. The diseases are: rheumatoid arthritis, juvenile rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, dermatomyositis and polymyositis. Enrollment is at the NIH Clinical Center in Bethesda, MD, or a local doctor’s office. There is no charge for study-related evaluations and medical tests. Reimbursement is available. For information, use the NIH Clinical Center information above or visit the clinicaltrials.gov web site.
A new unit of the National Institute of Environmental Health Sciences, called the Environmental Autoimmunity Group (EAG), has been established in Bethesda, MD, at the NIH to conduct pioneering research in understanding the genetic and environmental risk factors that may result in autoimmune disease.
The EAG is currently enrolling families in which an adult or child meets criteria for rheumatoid arthritis/juvenile rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, or myositis and in which a twin or sibling of the same gender, who is within 47 months of age, does not have any one of these four illnesses or another autoimmune disease. Subjects may enroll at the NIH Clinical Center in Bethesda or in their local doctors’ offices. Patients remain under the care of their personal physicians while participating in the study. There is no charge for study-related evaluations and medical tests at the NIH.
For information about the NIH Twin-Sibs Study, call 1-800-411-1222.
Evidence suggests that intravenous immunoglobulin (IVIG) therapy may significantly improve muscle strength, skin rash, and underlying, abnormal immune responses in some individuals with dermatomyositis resistant to or only partially responsive to standard therapies. Immune globulin is a concentrated preparation containing antibodies obtained from the fluid portion of the blood (plasma). Further studies are needed to determine the long-term safety and effectiveness (efficacy) of such therapy for individuals with dermatomyositis.
A pilot study evaluated the use of the immunosuppressive drug fludarabine in individuals with dermatomyositis or polymyositis who had insufficient response or unacceptable side effects to therapy with prednisone and at least one other standard immunosuppressive agent. Of 16 participants, four improved, seven were classified as unchanged, and five withdrew. According to the study’s investigators, such therapy was found to cause significant lymphopenia, or a decrease in the proportion of certain white blood cells, without an increase in complications due to infection over that associated with standard immunosuppressant medications used for individuals with myositis. Further research is needed to determine the long-term safety and effectiveness of such therapy for affected individuals.
A study is being sponsored by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) on the natural history and pathogenesis of dermatomyositis, polymyositis, and related diseases. The purpose of the study is to examine the causes of these diseases and describe associated symptoms and findings. Individuals who are age 16 years and older with dermatomyositis, polymyositis, or a related disease may be eligible for participation. For further information, contact:
National Institutes of Health (NIH)
Warren G. Magnuson Clinical Center
Patient Recruitment and Public Liaison Office
10 Cloister Court
Bethesda, MD 20892
Toll-free: (800) 411-1222
Home page: http://www.cc.nih.gov/
Web site for Clinical Center’s Protocol Database: http://clinicalstudies.info.nih.gov/
The Juvenile Dermatomyositis (JDM) Registry is a diagnosis-based national registry that was established in 1994. Funded by the NIAMS, it is based at the Children’s Memorial Hospital in Chicago, Illinois. The purpose of the Registry is to determine incidence and prevalence* of juvenile dermatomyositis, the circumstances surrounding disorder onset, and the influences that ethnic background may have on disease course as well as to facilitate research across the United States. (*Incidence refers to the number of new cases of a disorder occurring during a specific period, while prevalence indicates the total number of all old and new cases at any one time.) For more information, physicians with pediatric patients who have been diagnosed with juvenile dermatomyositis and have not been previously referred may contact:
Kathy Abbott, JDM Research Nurse
Toll-free: (888) 719-8098
Phone: (773) 880-3333
Fax: (773) 880-4179
Lauren Pachman, MD, Primary Investigator
Phone: (773) 880-4360
Division of Immunology/Rheumatology
Children’s Memorial Hospital
2300 Children’s Plaza
Chicago, IL 60614
Toll-free: (888) 719-8098
Web site: http://www.childrensmemorial.org/jdm/
A clinical trial is being sponsored by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) to determine the effectiveness of the drug infliximab (Remicade) for the treatment of dermatomyositis and polymyositis. Individuals 18 years of age and older with active dermatomyositis or polymositis that does not respond adequately to treatment with methotrexate and corticosteroids may be eligible for this study. For more information, contact:
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
9000 Rockville Pike
Bethesda, Maryland 20892
Patient Recruitment and Public Liaison Office
A clinical trial is underway to study the effectiveness of early introduction of etanercept or methotrexate in the treatment of juvenile dermatomyositis. The long-term goal is to improve the treatment of this serious childhood onset rheumatic disease and to better understand the pathogenic mechanism for the development of the vasculopathy (disorder of blood vessels) of JDMS. For more information: contact:
Cincinnati Children’s Hospital Medical Center
Cincinnati, Ohio 45229
Daniel J. Lovell, MD, MPH, Principal Investigator
Some of the organizations listed in the “Resources” section of this report below may be able to provide further information concerning clinical trials on dermatomyositis.
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eMedicine-Dermatomyositis: Article by Jeffrey P Callen, MD