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Disease Profile
Amyopathic dermatomyositis
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
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Age of onset
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ICD-10
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Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
ADM; Dermatomyositis sine myositis
Categories
Connective tissue diseases; Kidney and Urinary Diseases; Nervous System Diseases;
Summary
Amyopathic dermatomyositis is a form of dermatomyositis characterized by the presence of typical skin findings without muscle weakness.[1][2] Some of the skin changes that suggest dermatomyositis include a pink rash on the face, neck, forearms and upper chest; Gottron's papules and heliotrope eyelids.[2][3][4] Pruritis and photosensitivity are common, as is scalp inflammation and thinning of the hair.[2][3] While patients with amyopathic dermatomyositis should not have clinically evident muscle weakness, minor muscle abnormalities may be included. Fatigue is reported in at least 50% of patients.[2] Some cases have been associated with internal malignancy and/or interstitial lung disease.[5] Treatment may include sun avoidance, ample use of sunscreen, topical
Symptoms
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
80%-99% of people have these symptoms | ||
Abnormal eyelid morphology |
Abnormality of the eyelid
Abnormality of the eyelids
[ more ] |
0000492 |
Autoimmunity |
Autoimmune disease
Autoimmune disorder
[ more ] |
0002960 |
EMG abnormality | 0003457 | |
Erythema | 0010783 | |
Inflammatory myopathy | 0009071 | |
Myalgia |
Muscle ache
Muscle pain
[ more ] |
0003326 |
Periorbital edema | 0100539 | |
Proximal muscle weakness |
Weakness in muscles of upper arms and upper legs
|
0003701 |
30%-79% of people have these symptoms | ||
Abnormal hair quantity | 0011362 | |
Abnormality of the nail | 0001597 | |
Acrocyanosis |
Persistent blue color of hands, feet, or parts of face
|
0001063 |
Arthralgia |
Joint pain
|
0002829 |
Joint inflammation
|
0001369 | |
Chondrocalcinosis |
Calcium deposits in joints
|
0000934 |
Diffuse reticular or finely nodular infiltrations | 0002207 | |
Dry skin | 0000958 | |
Fatigue |
Tired
Tiredness
[ more ] |
0012378 |
Muscular |
Low or weak muscle tone
|
0001252 |
Papule | 0200034 | |
Pruritus |
Itching
Itchy skin
Skin itching
[ more ] |
0000989 |
Pulmonary fibrosis | 0002206 | |
Recurrent respiratory infections |
Frequent respiratory infections
Multiple respiratory infections
respiratory infections, recurrent
Susceptibility to respiratory infections
[ more ] |
0002205 |
Respiratory insufficiency |
Respiratory impairment
|
0002093 |
Skin ulcer |
Open skin sore
|
0200042 |
Weight loss | 0001824 | |
5%-29% of people have these symptoms | ||
Abnormal eosinophil morphology | 0001879 | |
Aplasia/Hypoplasia of the skin |
Absent/small skin
Absent/underdeveloped skin
[ more ] |
0008065 |
Breast carcinoma |
Breast cancer
|
0003002 |
Cellulitis |
Bacterial infection of skin
Skin infection
[ more ] |
0100658 |
Cutaneous photosensitivity |
Photosensitive skin
Photosensitive skin rashes
Photosensitivity
Sensitivity to sunlight
Skin photosensitivity
Sun sensitivity
[ more ] |
0000992 |
Dysphonia |
Inability to produce voice sounds
|
0001618 |
Feeding difficulties in infancy | 0008872 | |
Fever | 0001945 | |
Gangrene |
Death of body tissue due to lack of blood flow or infection
|
0100758 |
Gastrointestinal stroma |
0100723 | |
Lung adenocarcinoma | 0030078 | |
Lymphoma |
Cancer of lymphatic system
|
0002665 |
Myocardial infarction |
Heart attack
|
0001658 |
Myocarditis |
Inflammation of heart muscle
|
0012819 |
Pericarditis |
Swelling or irritation of membrane around heart
|
0001701 |
Pulmonary arterial |
Increased blood pressure in blood vessels of lungs
|
0002092 |
Sinus tachycardia | 0011703 | |
Telangiectasia of the skin | 0100585 | |
Vasculitis |
Inflammation of blood vessel
|
0002633 |
Related diseases
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
|
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The differential diagnoses include muscular dystrophies of late onset, as well as adult-onset nemaline myopathy, proximal myotonic myopathies and systemic lupus erythematosus, pityriasis rubra pilaris, lichen planus (see these terms), and polymorphous light eruption.
Visit the Orphanet disease page for more information.
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Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
-
Myositis UK
146 Newtown Road
Woolston
Southampton
SO19 9HR
United Kingdom
Telephone: 023 8044 9708
E-mail: [email protected]
Website: https://www.myositis.org.uk/ -
The Myositis Association (TMA)
1940 Duke Street
Suite 200
Alexandria, VA 22314
Toll-free: 1-800-821-7356
Telephone: +1-703-299-4850
Fax: +1-703-535-6752
E-mail: [email protected]
Website: https://www.myositis.org/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
In-Depth Information
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Amyopathic dermatomyositis. Click on the link to view a sample search on this topic.
References
- Callen JP. Dermatomyositis. Medscape Reference. October 2011; https://emedicine.medscape.com/article/332783-overview.
- Olsen NJ, Park JH, King LE Jr. Amyopathic dermatomyositis. Curr Rheumatol Rep. Aug 2001; https://www.ncbi.nlm.nih.gov/pubmed/11470054.
- Dermatomyositis. DermNet NZ. 2011; https://www.dermnetnz.org/immune/dermatomyositis.html.
- Saoud B, Allali F, Hassouni NH. Amyopathic dermatomyositis. Joint Bone Spine. May 2006; https://www.ncbi.nlm.nih.gov/pubmed/16253536.
- Sato S, Kuwana M. Clinically amyopathic dermatomyositis. Curr Opin Rheumatol. Nov 2010; https://www.ncbi.nlm.nih.gov/pubmed/20827200.