{
  "abstract": "Anti- melanocyte differentiation-associated protein 5 (MDA5) associated dermatomyositis is a rare, heterogeneous disease with multiple phenotypes. This case describes a patient with indolent myopathy, skin lesions and normal creatine kinase (CK). After a period of diagnostic uncertainty, the patient was found to have positive anti-MDA5 and anti-Ro52 antibodies, confirming anti-MDA5 dermatomyositis with cutaneous vasculopathy. Recognition of vasculopathy as a manifestation of dermatomyositis could have led to a much earlier diagnosis. The European Alliance of Associations for Rheumatology /American College of Rheumatology classification criteria can aid diagnosis. Antibody profile correlates with disease phenotype in dermatomyositis. Anti-MDA5 dermatomyositis is typically associated with rapidly progressive interstitial lung disease and amyopathic dermatomyositis. Some patients may present with myopathy despite a normal CK. Vasculopathic skin lesions are common, but frequently under-recognised, and can develop from Gottron’s papules. All phenotypes are associated with increased morbidity and mortality. This underscores the importance of recognising such vasculopathy as a sign of anti-MDA5-associated dermatomyositis, thus contributing to earlier diagnosis and treatment.",
  "authors": [
    {
      "affiliations": [
        "Kellgren Centre, Rheumatology, Manchester University NHS Foundation Trust, Manchester, Greater Manchester, UK"
      ],
      "name": "Martin Regan"
    },
    {
      "affiliations": [
        "Kellgren Centre, Rheumatology, Manchester University NHS Foundation Trust, Manchester, Greater Manchester, UK"
      ],
      "name": "Yee Ho Chiu"
    },
    {
      "affiliations": [
        "Manchester University NHS Foundation Trust, Manchester, England, UK"
      ],
      "name": "James Culley"
    },
    {
      "affiliations": [
        "Kellgren Centre, Rheumatology, Manchester University NHS Foundation Trust, Manchester, Greater Manchester, UK"
      ],
      "name": "Rikesh Patel"
    }
  ],
  "title": "Cutaneous clues to diagnosis: the vasculopathic form of anti-MDA5 dermatomyositis",
  "uid": "ce05a220-0828-54c8-b03a-ae2ed705376a"
}