MANIFESTATIONS OF THE SKIN IN DERMATOMYOSITIS AND THEIR DIAGNOSTIC SIGNIFICANCE: A LITERATURE REVIEW
DOI:
https://doi.org/10.31435/ijitss.2(50).2026.5370Keywords:
Dermatomyositis; Cutaneous Manifestations; Myositis-Specific Autoantibodies; Anti-MDA5; Interstitial Lung Disease; Shawl Sing; CDASI; anti-TIF1-γ; anti-NXP2, JAK Inhibitors; Clinically Amyopathic Dermatomyositis; Heliotrope Rash; Calcinosis Cutis; PoikilodermaAbstract
Background. Dermatomyositis (DM) is an idiopathic inflammatory myopathy in which characteristic cutaneous manifestations often precede or occur independently of muscle involvement. Skin findings are essential for diagnosis, phenotype classification and prognostic assessment, reflecting underlying immunopathologic processes.
Aim of the study. To review and summarize current evidence on the spectrum of cutaneous manifestations in dermatomyositis, their associations with myositis-specific autoantibodies, and implications for prognosis, monitoring and management.
Materials and methods. A narrative review of peer-reviewed literature was performed. Searches were conducted in PubMed and in the Medical University of Gdańsk (GUMed) online library catalog. Search terms included “dermatomyositis”, “cutaneous manifestations,” “autoantibodies” and related phenotype and outcome terms. Priority was given to recent cohort studies, key reviews and illustrative case series; articles in English and Polish were considered. Selected sources were synthesized qualitatively to describe clinical morphology, serologic correlations, validated skin assessment tools and therapeutic approaches.
Conclusions. Classic cutaneous signs (heliotrope rash, Gottron papules/sign, shawl and V-neck distributions, poikiloderma) remain central to DM recognition. Distinct skin phenotypes correlate with specific autoantibodies (e.g., anti-MDA5 with ulcerations and high ILD risk; anti-TIF1-γ with extensive eruptions and malignancy association; anti-NXP2 with calcinosis). Standardized scoring (CDASI) aids objective assessment. Integrating detailed dermatologic evaluation with antibody profiling improves early diagnosis, risk stratification and individualized management. Prospective, phenotype-driven studies and refinement of classification criteria-particularly to better capture amyopathic variants-are needed.
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Copyright (c) 2026 Sylwia Skraińska, Agnieszka Kiedik, Julia Kozłowska, Kacper Zima, Zuzanna Pietruk, Jakub Chamier-Gliszczyński, Dominik Płaza, Przemysław Siemiątkowski
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