JAMA DERMATOLOGY PATIENT PAGE
Dermatomyositis
Dermatomyositis (DM) is a condition affecting the skin (dermato) and muscles (myositis).
Cutaneous DM is an inflammatory disease with characteristic skin
Dermatomyositis is an inflammatory disease with characteristic skin changes
findings and frequent inflammation of the muscles (myositis). Pa- and muscle inflammation (myositis).
tients may also have internal disease that can affect the lungs, joints, Heliotrope rash around the eyes Gottron papules
esophagus, and heart. Dermatomyositis can occur in children or on the hand
adults. This disease in children may result in calcium deposits in the
skin. Adults with DM could have an internal cancer. Some patients
have only skin disease, which is called amyopathic DM, while oth-
ers have skin changes and myositis. In some patients, the skin dis-
ease may still be active even if the myositis is effectively treated. The
cause of DM is not known, but it might be associated with inherited
factors, reaction to an infectious agent, a medication, sunlight, or
internal cancer. Rash on the upper chest
Symptoms and Manifestations
The classic skin changes in DM include a heliotrope rash (a red-to-
violet–colored rash around the eyes that might be accompanied by Shawl sign on
swelling) and Gottron papules (red bumps most prominently seen the upper back
on the knuckles of the hands). Other characteristic skin findings in-
clude red purplish discoloration on the face, exposed surfaces of the
arms, forearms and hands, scalp, upper chest, upper back (known
as a Shawl sign), and the lateral hips (known as the holster sign) in Muscle inflammation typically affects muscles of the shoulder
and hip. Internal organs may be affected, including disease
addition to changes around the cuticles of the fingernails. Many pa- of the esophagus, lungs, and less commonly the heart.
tients are sensitive to sunlight. The muscle inflammation tends to
affect larger muscles and can present as difficulty raising the arms
above the shoulder or trouble rising from a chair or climbing stairs. The skin must be treated with sun-protective measures, includ-
Sometimes there is muscle tenderness, fatigue, and low-grade fe- ing avoidance of sunlight, use of protective clothing, and sun-
ver. Dermatomyositis can also affect the esophagus (followed by dif- screens. Topical therapies, including creams and ointments, may be
ficulty swallowing), lungs (followed by shortness of air), or heart. prescribed, but are unlikely alone to effectively treat skin DM. Most
patients need long-term use of systemic medications, including im-
Diagnosis and Evaluation munosuppressants (given as a pill), or a medication given as an in-
A physician will perform a thorough skin examination, test your fusion called immune globulin. Response to treatment is assessed
strength, take a thorough history, order blood tests, perform a skin by physical examination of the skin and muscles and for muscle dis-
biopsy, and possibly perform tests or imaging of your muscles. If you ease by repeated blood testing.
are an adult, you will need to be screened for internal cancer at the
time of diagnosis, and these tests are usually repeated annually for
3 years. In addition, the function of your esophagus, lungs, and heart FOR MORE INFORMATION
will also be evaluated. The Myositis Association
https://www.myositis.org/
Treatment National Institute of Neurological Disorders and Stroke
Management of DM is aimed at the organs affected. Patients with https://www.ninds.nih.gov/health-information/disorders/
muscle disease are usually treated with oral corticosteroids (pred- dermatomyositis
nisone) initially, with transitioning to use of other systemic medica- National Organization for Rare Disorders
tions that lower inflammation. Physical therapy to maintain the full https://rarediseases.org/rare-diseases/dermatomyositis/
range of motion of the joints and prevent muscle loss is helpful.
Author: Jeffrey P. Callen, MD The JAMA Dermatology Patient Page is a public service of JAMA Dermatology. The
Published Online: July 5, 2023. doi:10.1001/jamadermatol.2023.1013 information and recommendations appearing on this page are appropriate in most
instances, but they are not a substitute for medical diagnosis. For specific information
Author Affiliation: Division of Dermatology, University of Louisville, Louisville, concerning your personal medical condition, JAMA Dermatology suggests that you
Kentucky. consult your physician. This page may be downloaded or photocopied
Conflict of Interest Disclosures: None reported. noncommercially by physicians and other health care professionals to share with
patients. To purchase bulk reprints, email [email protected].
Section Editor: Courtney Schadt, MD.
jamadermatology.com (Reprinted) JAMA Dermatology Published online July 5, 2023 E1
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