Perivascular and interstitial dermatosis in anti-HMGCR immune-mediated necrotizing myopathy: A case report


Topic:

Clinical Management

Poster Number: 111 S

Author(s):

Michael Arias, DO, MPH, UCLA David Geffen School of Medicine, Tina Chen, MD, Sharp Healthcare, San Diego, Razieh Soltani-Arabshahi, MD, Pasadena Premier Dermatology, Los Angeles, Taylor Deal, MD, Sharp Memorial Hospital, San Diego, Payam Soltanzadeh, MD, University of California, Los Angeles (UCLA)

This is the case of a 56-year-old man who presented with severe leg weakness, violaceous indurated truncal rash, and hyperCKemia (creatine kinase (CK) of > 26000 at time of initial consult). He was diagnosed with statin-triggered anti-HMGCR immune-mediated necrotizing myopathy (HMGCR IMNM), and immunotherapy with IVIG, steroids, and rituximab significantly helped with his myopathy and skin rashes. Later in the course of his disease (about 2.5 years later), CK levels began to rise again accompanied by reappearance and expansion of the rash involving the trunk as well as the shoulders and upper arms. Skin biopsy revealed superficial and deep interstitial, perivascular, and perieccrine mixed inflammation with mostly lymphocytes and neutrophils consistent with an autoimmune eruption. Escalation of immunotherapy helped with partial resolution of the rash and IMNM. Dermatomyositis-like rashes have been described in HMGCR IMNM; however, this patient’s skin manifestations are distinct and seem to correlate with the severity of muscle involvement. Dermatologists and neurologists should be aware of this association and its clinical implications to guide optimal management.