NXP-2 Positive Dermatomyositis: A Unique Clinical Presentation. (13th June 2017)

• Record Type: Journal Article
• Authors: Butt, Zeeshan; Patel, Leeza; Das, Manash K.; Mecoli, Christopher A.; Ramji, Alim
• Other Names: Cevik Remzi Academic Editor.
• Is Part Of: Case reports in rheumatology. Volume 2017(2017)
• Publication Date: 2017-06-13
• Subjects: Rheumatology -- Periodicals; Rheumatology -- Case studies -- Periodicals; Rheumatic Diseases; Connective Tissue Diseases; Rheumatology; Periodicals; Electronic journals; Case studies; Periodicals; 616.723
• DOI: 10.1155/2017/4817275
• Languages: English
• ISSNs: 2090-6889

Abstract

Abstract : 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.