# Anti-nuclear Matrix Protein 2 Antibody-Positive Dermatomyositis Relapse With Preceding Panniculitis

## TL;DR

A case of relapsing dermatomyositis with panniculitis and anti-NXP-2 antibodies is reported, highlighting panniculitis as a rare skin manifestation.

## Contribution

This case highlights panniculitis as a rare cutaneous manifestation of DM relapse without muscle symptoms.

## Key findings

- Panniculitis can be an initial sign of DM relapse even without muscle symptoms.
- Anti-NXP-2 antibody positivity was observed in the patient during relapse.
- Treatment with PSL and tacrolimus resolved the symptoms.

## Abstract

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by cutaneous lesions such as heliotrope rash, Gottron's papules, and Gottron's sign. Panniculitis is rarely reported as a skin manifestation of DM. Herein, we present a case of anti-nuclear matrix protein 2 antibody (NXP-2)-positive DM relapse with panniculitis in the absence of muscle symptoms. A 32-year-old female patient was referred to our department. Nine years ago, she was diagnosed with DM and had received prednisolone (PSL) and tacrolimus therapy. However, she discontinued her visits four years ago. She most recently presented with erythematous lesions on the right thigh and buttocks without any muscle symptoms. Thirteen days later, she experienced upper limb weakness. Laboratory findings at her current presentation demonstrated elevated serum creatine kinase levels and positivity for anti-NXP-2 antibody. A skin biopsy of the erythema on her left thigh demonstrated findings consistent with healed panniculitis. Based on these findings, an anti-NXP-2 antibody-positive DM relapse was diagnosed. Her symptoms resolved with PSL and tacrolimus therapy. It is important to recognize that panniculitis is a significant, cutaneous manifestation of DM and can present as the initial manifestation of a DM relapse even in the absence of muscle symptoms.