A case of necrotising autoimmune myopathy with inflammatory skin lesions

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Necrotising autoimmune myopathy (NAM) is caused by immune system deregulation. The inflammatory component is generally very low key, contrarily to the other types of autoimmune myositis. It is, to a large extent, associated with the presence of autoantibodies directed against SRP and/or HMG-CoA Reductase (HMGCR) proteins.

An observation from Brazil relates the combination of NAM with erythema-type skin signs in a young female 8-year-old patient:

  • the muscle biopsy showed an appearance of necrosis and an increase in HLA class I labelling;
  • the skin biopsy showed perivascular inflammatory lesions;
  • the anti-SRP and anti-HMGCR antibodies were negative, whereas CPK levels were very high.

 

Immune-Mediated Necrotizing Myositis Presenting with Cutaneous Lesions. Souza BCE, Fasciani IA, Swiczar BCC et al. Indian J Dermatol. 2021 Jul-Aug;66(4):447.

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