NXP-2 Positive Dermatomyositis: A Unique Clinical Presentation

Abstract EN

Dermatomyositis (DM), a myopathy associated with inflammation and muscle weakness, has historically been difficult to diagnose.

Recently, nuclear matrix protein (NXP-2) antibodies have been described as a myositis-specific antibody that may aid in the diagnostic evaluation.

We present the case of a 21-year-old, previously healthy, African American male with DM.

He presented to our outpatient clinic with periorbital swelling and a rash, for which he was started on prednisone by an ophthalmologist.

Towards the end of the prednisone taper, he began to experience muscle weakness, a worsening rash, and dysphagia to solids with a resultant loss of 60 pounds within a month.

He was transferred to a tertiary care hospital where he was further evaluated and ultimately diagnosed with dermatomyositis, supported by skin and muscle biopsies, and was found to be positive for NXP-2.

He was given intravenous immunoglobulin (IVIG) and high-dose steroids with improvement.