$Unique_ID{BRK03659} $Pretitle{} $Title{Dermatomyositis} $Subject{Dermatomyositis Childhood Dermatomyositis Primary Idiopathic Dermatomyositis in Adults Dermatomyositis malignant tumors Dermatomyositis connective tissue disease overlap syndromes Sclerodermatomyositis Mixed Connective Tissue disease} $Volume{} $Log{} Copyright (C) 1986, 1990, 1992, 1993 National Organization for Rare Disorders, Inc. 262: Dermatomyositis ** IMPORTANT ** It is possible the main title of the article (Dermatomyositis) is not the name you expected. Please check the SYNONYMS listing to find the alternate names covered by this article. Synonyms Childhood Dermatomyositis Primary Idiopathic Dermatomyositis in Adults Dermatomyositis associated with malignant tumors Dermatomyositis associated with connective tissue disease overlap syndromes, including Sclerodermatomyositis and Mixed Connective Tissue disease General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Dermatomyositis is a systemic connective tissue disorder characterized by inflammatory and degenerative changes in the muscles and in the skin. Symptoms Symptoms of Dermatomyositis may start gradually or suddenly. The symptoms often wax and wane for no apparent reason. The major symptom of the disorder is muscle weakness, most often in the hip and shoulder areas, eventually making it difficult for patients to lift their arms or to climb steps. Other muscles which may be affected are the neck and throat muscles, which may result in difficulty swallowing and cause changes in the voice. Rarely, chest muscles may be affected. The muscle weakness may appear suddenly and progress over weeks to months. Difficulty in swallowing and dilatation of the lower esophagus and small intestine may be indistinguishable from that in Scleroderma (For more information on Scleroderma, choose "scleroderma" as your search term in the Rare Disease Database.) The muscles of the hands, feet and face often escape involvement. Contractures of the limbs may eventually develop. The person with Dermatomyositis may develop a patchy, reddish skin rash on the face, around the eyes, on the knuckles and elbows and sometimes on the knees and ankles. The skin lesions frequently fade completely but may be followed by brownish pigmentation, atrophy, scarring or loss of pigmentation (vitiligo). (For more information, choose "vitiligo" as your search term in the Rare Disease Database.) Some people also experience puffy eyelids. Other symptoms may include fever, weight loss and occasionally pain or tenderness in muscles and joints. A few people with Dermatomyositis have an extreme sensitivity to cold that is most often felt in the fingers (Raynaud's Phenomenon). This is caused by spastic narrowing of blood vessels in the fingers. (For more information, choose "Raynaud" as your search term in the Rare Disease Database). People with Dermatomyositis may also develop numb and shiny red areas around and under the finger nails. The characteristic skin changes in this disorder can suggest scleroderma in some patients. Subcutaneous calcification may occur, particularly in childhood Dermatomyositis. This is similar in distribution to that encountered in Scleroderma, but tends to be more extensive (calcinosis universalis), particularly in untreated or undertreated patients. Pain in many joints (polyarthralgia), accompanied at times by swelling, fluid and other evidence of non-deforming arthritis, occurs in approximately one third of patients with dermatomyositis. These rheumatic complaints tend to be mild and respond well to corticosteroids. Gastrointestinal involvement, except for the pharynx and the esophagus, is relatively uncommon in Dermatomyositis. Inflammation of the lungs with an increase of interstitial tissue (interstitial pneumonitis) is manifested by difficulty breathing and by coughing. These respiratory symptoms may precede myositis and dominate the clinical picture. Involvement of the heart, detected chiefly by irregularities in the electrocardiogram (ECG), has been reported. Acute kidney failure can occur as a consequence of excess muscle protein "myoglobin" in the urine (Crush syndrome) due to severe acute disintegration of muscle (rhabdomyolysis). Sjogren syndrome can also occur in some patients with Dermatomyositis. (For more information, choose "Sjogren" as your search term in the Rare Disease Database). Abdominal symptoms, more common in children, may be associated with the passage of dark stools or the vomiting of blood from gastro-intestinal ulcers that may progress to perforation and require surgical intervention. An associated malignancy, usually a carcinoma, may occur in about 15% of men and a smaller proportion of women over age 50 with Dermatomyositis. Causes The cause of Dermatomyositis is unknown. The disorder may be caused by the body's natural immune defense mechanisms attacking its own healthy tissue (autoimmune reaction). Viruses may also play a role. Affected Population Dermatomyositis may appear at any time from infancy through age 80, but most commonly it occurs between 40 to 60 years. In children, the symptoms usually appear between the ages of 5 to 15 years. Females are affected twice as often as males. Related Disorders Scleroderma (Progressive Systemic Sclerosis) is a rare, chronic collagen vascular disorder characterized by diffuse hardening, degenerative changes and vascular inflammation of the connective tissues of the skin, joints and many visceral organs. It shares certain clinical findings with dermatomyositis. Systemic Lupus Erythematosus (SLE) is an inflammatory connective tissue disorder that can affect many parts of the body including the joints, skin and internal organs. SLE is a disease of the body's immune system. It shares certain clinical findings with dermatomyositis. Polymyositis is a form of a systemic connective tissue disorder characterized by inflammatory and degenerative changes in the muscles, leading to weakness and some degree of muscle atrophy. The areas principally affected are the hip, shoulder and chest muscles. (For more information on these related disorders, choose "Scleroderma", "Lupus", or "Polymyositis" as your search terms in the Rare Disease Database.) Therapies: Standard Corticosteroids such as prednisone, together with antacids and potassium supplements, are widely used in treatment of dermatomyositis. Measurement of muscle enzyme activity is used to gauge the effectiveness of therapy. Reduction of these enzymes to normal values is noted in a majority of patients with this disorder within 4 to 6 weeks after treatment is started. This is followed by an improvement in muscle strength. At this point the dose of prednisone can usually be reduced slowly. In many cases of adult dermatomyositis prolonged maintenance therapy with prednisone may be necessary. Children with dermatomyositis may be able to discontinue prednisone after a year or more, experiencing apparent remission. Immunosuppressive drugs such as methotrexate, cyclophosphamide, chlorambucil and azathioprine have been beneficial to some patients who fail to respond to corticosteroids alone. Some patients have received methotrexate for 5 years or longer for control of this disorder. Therapies: Investigational The FDA has approved testing of the orphan product Immune Globulin Intravenous Human (Iveegan, Immuno) for the disease Dermatomyositis. The drug is sponsored by Iuumno Clinic Research Corp., New York, NY. Cyclosporine (Sandimmune) may be of potential benefit for treating a number of dermatologic diseases. These include Pemphigus and Bullous Pemphigoid, Posterior Uveitis and Behcet's Syndrome, collagen vascular disorders such as severe Dermatomyositis, Sjogren's Syndrome, and Scleroderma, Mycosis Fungoides, and Alopecia Areata. Certain types of skin grafts have also shown improvement after cyclosporine treatment, in some cases. However, this drug may also be associated with severe and life- threatening side effects which would limit its use in many patients. Careful monitoring of this drug by a physician is necessary to guard against possible toxic side effects. Relapses can occur when the drug is discontinued. More research is needed before cyclosporine can be recommended as a treatment for all but the most severe cases of the disorders listed above. Even for the most severe cases its use is still experimental, and long-term effects are unknown. Studies are being conducted in the use of Sandoglobulin as a treatment for Dermatomyositis. Further investigation is needed to determine it's safety and effectiveness. This disease entry is based upon medical information available through March 1993. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Dermatomyositis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Dermatomyositis and Polyomyositis Support Group 146 Newtown Rd. Woolston, Southhampton SO2 9HR England Phone Southhampton 449708 Arthritis Foundation 1314 Spring Street, N.W. Atlanta, GA 30309 (404) 872-7100 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References POLYMYOSITIS AND DERMATOMYOSITIS: C. M. Pearson; Arthritis Medical Information Series, Arthritis Foundation, 1983. THE MERCK MANUAL 15th ed: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. P. 1280. CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. Pp. 1978, 2341.