A rare case of NXP-2 positive dermatomyositis.

Dermatomyositis is an idiopathic inflammatory myopathy with variable cutaneous manifestations. Several autoantibodies each with distinct clinical phenotypes are associated with the disease. Here we present the case of a 36-year-old Laotian woman with hypothyroidism who presented with severe proximal and distal muscle weakness, dysphagia, diffuse rash, and anasarca that was diagnosed with NXP-2 (nuclear matrix protein 2) antibody positive dermatomyositis. The patient's hospitalization was complicated by disease resistant to conventional therapy.