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eISSN: 2084-9893
ISSN: 0033-2526
Dermatology Review/Przegląd Dermatologiczny
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2/2021
vol. 108
 
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abstract:
Special paper

Dermatomyositis. Diagnostic and therapeutic recommendations of the Polish Dermatological Society

Adam Reich
1
,
Anna Lis-Święty
2
,
Dorota Krasowska
3
,
Joanna Maj
4
,
Joanna Narbutt
5
,
Anna Sysa-Jedrzejowska
6
,
Anna Wojas-Pelc
7
,
Anna Woźniacka
6
,
Lidia Rudnicka
8

  1. Department of Dermatology, Institute of Medical Sciences, Medical College of Rzeszow University, Poland
  2. Department of Dermatology, Venereology and Pediatric Dermatology, Medical University in Lublin, Poland
  3. Department of Dermatology, Silesian Medical University, Katowice, Poland
  4. Department of Dermatology, Venereology and Allergology, Wroclaw Medical University, Poland
  5. Department of Dermatology, Pediatric and Oncological Dermatology, Medical University of Lodz, Poland
  6. Department of Dermatology and Venereology, Medical University of Lodz, Poland
  7. Department of Dermatology and Venereology, Jagiellonian University Medical College, Krakow, Poland
  8. Department of Dermatology, Warsaw Medical University, Poland
Dermatol Rev/Przegl Dermatol 2021, 108, 85-104
DOI: https://doi.org/10.5114/dr.2021.107278
Online publish date: 2021/06/28
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Dermatomyositis is an autoimmune disease characterized by skin lesions and/or symptoms of myositis. In addition to the so-called classic dermatomyositis the following forms of dermatomyositis are distinguished based on the clinical presentation: pediatric, paraneoplastic, drug-induced and amyopathic. A number of disease-specific autoantibodies are identified in dermatomyositis (including anti-Mi2, anti-TIF1, anti-NXP2, anti-SAE or anti-MDA5), presence of which may be associated with a specific clinical phenotype. The diagnosis of the severity of muscle involvement is currently based mainly on physical examination, deviations in results of laboratory investigations, electromyographic examination and imaging examination, mainly with the use of magnetic resonance imaging. Systemic glucocorticosteroids administered as monotherapy or in combination with other immunosuppressants remain the mainstay of dermatomyositis treatment, and in the absence of satisfactory improvement, intravenous immunoglobulins are used. In addition, in case of interstitial lung disease, the use of cyclophosphamide may be necessary. The choice of a therapy, as well as the rate of dose reduction, depend on the dynamics of the disease, symptoms, diagnosed immunological disorders, as well as comorbidities and the drugs used. Each diagnostic and therapeutic decision must take into account the individual clinical data of the patient and current scientific reports.
keywords:

dermatomyositis, idiopathic inflammatory myopathies, autoimmune diseases of connective tissue, treatment
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