Summary
Definition
History and exam
Key diagnostic factors
- Gottron papules
- heliotrope rash with or without periorbital edema
- macular violaceous erythema
- periungual erythema, nail-fold capillary dilation, cuticular overgrowth
- "mechanic's" hands
- proximal muscle weakness
Other diagnostic factors
- photosensitivity
- poikiloderma vasculare atrophicans
- pruritus
- fatigue and malaise
- dyspnea
- weight loss
- fever
- myalgia
- arthralgia
- dysphagia
- palpitations and syncope
- Raynaud phenomenon
- cutaneous calcinosis
- erythroderma
- vesiculobullous lesions
- leukocytoclastic vasculitis
- cutaneous necrosis
- nonscarring alopecia
Risk factors
- genetic predisposition
- bimodal age distribution: children and age >40 years
- female sex
- black race
- ultraviolet radiation
- infectious agents
- drugs
- immunization
Diagnostic tests
1st tests to order
- serum creatine kinase (CK)
- serum aldolase
- muscle biopsy
- electromyogram (EMG)
- skin biopsy
- antinuclear antibody (ANA)
- myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs)
Tests to consider
- muscle MRI
- ECG
- echocardiography
- cardiac troponin I
- chest x-ray (CXR)
- pulmonary function tests (PFTs)
- high-resolution CT of chest
- barium swallow or videofluoroscopic assessment of swallow
Treatment algorithm
severe disease or acute flares
combined muscle and skin disease
skin disease only
Contributors
Authors
Michael Edward Farrugia, MD, PgDip, MRCP (Derm)
Dermatology Specialty Trainee
Lauriston Building
NHS Lothian
Edinburgh
UK
Disclosures
MEF has received funding from AbbVie to attend an international dermatology conference.
Meng May Chee, MBChB, MD, MRCP
Consultant Rheumatologist
Queen Elizabeth University Hospital
Glasgow
UK
Disclosures
MMC accepted a sponsored invitation to attend the EULAR e-congress in June 2020.
Girish Gupta, FRCP
Consultant Dermatologist
Lauriston Building
Edinburgh
UK
Disclosures
GG has received honoraria for membership of advisory boards from Almirall, Leo, Lilly, Mylan, and Novartis. GG has also received lecture fees from AbbVie, Almirall, Galderma, La Roche Posay, Leo, Mylan, and Novartis. GG declares that none of the payments are related to this topic or treatments discussed in this topic, and there are no contractual agreements to disseminate product information.
Acknowledgements
Dr Michael E. Farrugia, Dr Meng May Chee, and Dr Girish Gupta would like to gratefully acknowledge Dr Sarah A. Saunders, Dr Rajan Madhok, and Dr Nicola Alcorn, previous contributors to this topic.
Disclosures
SAS and NA declare that they have no competing interests. RM declared holding shares in GSK to the value less than £12,000.
Peer reviewers
Nizar Souayah, MD
Assistant Professor of Neurology
Director of EMG Laboratory & Peripheral Neuropathy Center
Department of Neurology & Neurosciences
New Jersey Medical School
Newark
NJ
Disclosures
NS declares that he has no competing interests.
Sami Khella, MD
Physician
Department of Neurology
Penn Presbyterian Medical Center
University of Pennsylvania Health System
Philadelphia
PA
Disclosures
SK declares that he has no competing interests.
Yusuf Yazici, MD
Assistant Professor
Associate Director
Arthritis Program
Director
Behcet's Syndrome Center
Seligman Center for Advanced Therapeutics
New York University Hospital for Joint Diseases
New York
NY
Disclosures
YY has served as a consultant for Bristol-Myers Squibb, Roche, Celgene, Centocor, and UCB, and has been a speaker for Pfizer and Bristol-Myers Squibb.
Differentials
- Subacute cutaneous lupus erythematosus (SCLE)
- Psoriasis
- Cutaneous T-cell lymphoma
More DifferentialsGuidelines
- 2023 American College of Rheumatology (ACR) guideline for the screening and monitoring of interstitial lung disease in people with systemic autoimmune rheumatic disease
- 2023 American College of Rheumatology (ACR) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic disease
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