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$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.