Statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like features: A case report

Abstract

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. This entity is not commonly known among dermatologists as there are usually no extramuscular manifestations. We report a rare case of statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like cutaneous features. The possibility of anti-HMGCR immune-mediated necrotizing myopathy should be considered in patients with cutaneous dermatomyositis-like features associated with severe proximal muscle weakness, highly elevated creatine kinase levels and possible statin exposure. This indicates the importance of muscle biopsy and specific autoantibody testing for accurate diagnosis, as well as significant therapeutic implications.

Dermatomyositis-like rash associated with anti-3-hydroxy-3-methylglutaryl-coenzyme a reductase autoantibody necrotizing myopathy following COVID-19 infection and vaccination

“…1,8,9 In addition, while rare, anti- HMGCR antibodies have been reported in other idiopathic inflammatory myopathies (IIM) and connective tissue diseases, including 1.9% of adult-onset DM cases, 6.7% of juvenile DM cases, and 1.2% of primary Sjögren's syndrome cases. 2,[9][10][11] The typical presentation of IMNM associated with anti-HMGCR antibodies is associated with a mean age of onset of 55 with a female predominance of acute to subacute onset of progressive proximal extremity weakness, more pronounced in the lower extremities. CK is elevated at least 10 times the upper limit of normal and remains elevated despite stopping statin therapy if the patient was previously exposed.…”

“…Gottron's papules, Gottron's sign, or heliotrope rash). 2,[4][5][6]10 This clinical entity has been recognized in the most recent 2018 ENMC dermatomyositis classification criteria as "anti-HMGCR myopathy with a DM-like rash" with a single case series comparing these patients against those with HMGCR antibodies without a DM-like rash and noting those with DM-like rashes had a younger age of onset and shorter disease duration at time of diagnosis. 13,16 In addition to the unique skin findings, the significance of the temporal relation to the patient's second COVID-19 vaccination and her prior COVID-19 infection remains uncertain.…”

“…The clinical presentation of immune-mediated necrotizing myopathies (IMNM) includes the acute or subacute onset of severe, symmetric proximal weakness with potential for facial weakness and/or dysphagia, elevated creatinine kinase (CK), predominant myofiber necrosis with minimal inflammation on muscle biopsy, and minimal or no extramuscular manifestations. [1][2][3] Recently, there have been rare case reports of patients presenting with a characteristic dermatomyositis (DM)type rash, including a heliotrope rash or Gottron's papules, who were subsequently found to have serologic evidence of 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies and muscle biopsies most consistent with IMNM as opposed to perifascicular atrophy and predominant perivascular perimysial inflammation. 2,[4][5][6] Here we present an additional case of a 62-year-old female who presented with a characteristic heliotrope and bimalar rash who was subsequently found to have HMGCRassociated necrotizing myopathy temporally associated with her 2 nd mRNA COVID-19 vaccination and remote history of statin exposure.…”

Anti-HMGCR myopathy: clinical and histopathological features, and prognosis

“…HMGCR-IMNM patients with skin conditions usually develop pruritic erythematous papules with photosensitivity (Fig. 1E, F) [28 ▪ ,29 ▪ ,50] and sometimes show the dermatological presentations of Jessner-Kanoff disease or cutaneous lymphoma [26 ▪ ,27 ▪ ]. However, the incidence of anti-HMGCR antibody in Jessner-Kanoff disease or cutaneous lymphoma is still unknown.…”

“…For HMGCR-IMNM patients, the frequency of extramuscular manifestations was low. However, several studies have reported skin conditions in these patients, including Raynaud phenomenon, a nonspecific skin rash and dermatomyositis-like skin eruptions [11,15,26 ▪ –29 ▪ ,50]. HMGCR-IMNM patients with skin conditions usually develop pruritic erythematous papules with photosensitivity (Fig.…”