1. To describe a unique patient with a heliotrope rash in the setting of Anti-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) myopathy.

    

2. To summarize the phenotypic spectrum of dermal manifestations associated with anti-HMGCR myopathy, which in turn may allow for earlier diagnosis and better characterization of the disease.

Anti-HMGCR myopathy is a type of idiopathic inflammatory myopathy characterized by proximal muscle weakness, elevated creatinine kinase (CK) levels, and anti-HMGCR antibodies. The disease most frequently affects women over 40 years of age that have previously used statins. The phenotypic spectrum of dermal manifestations of anti-HMGCR myopathy is yet to be elucidated and may allow for earlier diagnosis as well as provide insights into the disease characteristics.

We describe the case of a middle-aged female who was diagnosed with anti-HMGCR myopathy after she presented with 3 months of progressive proximal muscle weakness associated with a periorbital violaceous hue and edema. Our case is discussed in the context of a Pubmed-based literature review.  

1. Cutaneous stigmata are seen in more than 40% patients with Anti- HMGCR myopathy and may represent a distinct clinical subset of the disease.

    

2. Early identification of dermal manifestations of Anti-HMGCR myopathy may aid in faster diagnosis and may unlock insights into characteristics of the disease.