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Disease Profile

Amyopathic dermatomyositis

Prevalence
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.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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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

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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.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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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.

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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.

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Not applicable

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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 corticosteroids, antimalarial agents, methotrexate, mycophenolate mofetil, or intravenous (IV) immunoglobulin.[1]

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
Arthritis
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 hypotonia
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 tumor
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 hypertension
Increased blood pressure in blood vessels of lungs
0002092
Sinus tachycardia
0011703
Telangiectasia of the skin
0100585
Vasculitis
Inflammation of blood vessel
0002633

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

    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

        1. Callen JP. Dermatomyositis. Medscape Reference. October 2011; https://emedicine.medscape.com/article/332783-overview.
        2. Olsen NJ, Park JH, King LE Jr. Amyopathic dermatomyositis. Curr Rheumatol Rep. Aug 2001; https://www.ncbi.nlm.nih.gov/pubmed/11470054.
        3. Dermatomyositis. DermNet NZ. 2011; https://www.dermnetnz.org/immune/dermatomyositis.html.
        4. Saoud B, Allali F, Hassouni NH. Amyopathic dermatomyositis. Joint Bone Spine. May 2006; https://www.ncbi.nlm.nih.gov/pubmed/16253536.
        5. Sato S, Kuwana M. Clinically amyopathic dermatomyositis. Curr Opin Rheumatol. Nov 2010; https://www.ncbi.nlm.nih.gov/pubmed/20827200.