NXP-2 Positive Dermatomyositis: A Unique Clinical Presentation

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...

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Bibliographic Details
Main Authors: Zeeshan Butt, Leeza Patel, Manash K. Das, Christopher A. Mecoli, Alim Ramji
Format: Article
Language:English
Published: Hindawi Limited 2017-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2017/4817275
Description
Summary: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.
ISSN:2090-6889
2090-6897