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Myasthenia Gravis: Overview and Treatment01:20

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Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
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Anti-nuclear Matrix Protein 2 Antibody-Positive Dermatomyositis Relapse With Preceding Panniculitis.

Hirokazu Taguchi1, Yoshitaka Ueda1, Yohya Shigehara2

  • 1Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center, Fuchu, JPN.

Cureus
|March 10, 2025
PubMed
Summary

Panniculitis, a rare skin condition, can signal a Dermatomyositis (DM) relapse, even without muscle symptoms. This case highlights NXP-2 antibody-positive DM presenting with panniculitis, emphasizing its importance in diagnosis.

Keywords:
anti-nuclear matrix protein 2 antibodydermatomyositislipoatrophymyositis-specific antibodypanniculitisskin biopsy

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Area of Science:

  • Rheumatology
  • Dermatology
  • Immunology

Background:

  • Dermatomyositis (DM) is an idiopathic inflammatory myopathy.
  • Characteristic skin lesions include heliotrope rash, Gottron's papules, and Gottron's sign.
  • Panniculitis is an uncommon cutaneous manifestation of DM.

Observation:

  • A 32-year-old female with a history of DM presented with thigh and buttock erythema.
  • She had no initial muscle symptoms, but developed upper limb weakness 13 days later.
  • Skin biopsy revealed healed panniculitis.

Findings:

  • The patient was positive for anti-nuclear matrix protein 2 (NXP-2) antibody.
  • Elevated serum creatine kinase levels were noted.
  • Diagnosis of anti-NXP-2 antibody-positive DM relapse was confirmed.

Implications:

  • Panniculitis can be a significant cutaneous manifestation of DM.
  • It may herald a DM relapse even without concurrent muscle involvement.
  • Recognizing panniculitis is crucial for timely diagnosis and management of DM relapses.