A rare case of NXP-2 positive dermatomyositis

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Dermatomyositis is an idiopathic inflammatory myopathy characterized by muscle weakness and skin rashes.
• Specific autoantibodies are associated with distinct clinical presentations in dermatomyositis patients.

Observation:

• A 36-year-old Laotian woman with hypothyroidism presented with severe proximal and distal muscle weakness, dysphagia, a diffuse rash, and anasarca.
• The patient was diagnosed with NXP-2 (nuclear matrix protein 2) antibody-positive dermatomyositis.

Findings:

• The patient's condition was resistant to conventional therapies during hospitalization.
• This case underscores the variable and potentially severe manifestations of NXP-2 antibody-associated dermatomyositis.

Implications:

• This case emphasizes the importance of identifying specific autoantibodies, such as NXP-2, for accurate dermatomyositis diagnosis and management.
• Further research into treatment strategies for refractory NXP-2 positive dermatomyositis is warranted.