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Myopathy in a 61-year-old Hispanic man
Gary Parizher1, Timothy J Brown1, Mary Hon1
1Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
BMJ Case Reports
|April 19, 2019
Summary
A heliotrope rash, a dermatomyositis feature, led to a misdiagnosis in a patient with necrotizing autoimmune myositis. Prompted by this case, a broad differential diagnosis is crucial for immune-mediated myopathies.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Immune-mediated myopathies present with diverse clinical features, sometimes mimicking other conditions.
- Dermatomyositis (DM) is characterized by specific dermatological findings, including the heliotrope rash.
- Accurate diagnosis is essential for effective treatment and management of autoimmune myopathies.
Observation:
- A 61-year-old man presented with subacute weakness, heliotrope rash, and dysphagia, initially suspected as DM.
- Physical examination findings were inconsistent with DM, prompting further investigation.
- Magnetic resonance imaging (MRI) and muscle biopsy revealed necrotizing autoimmune myositis (NAM).
Findings:
- The patient was diagnosed with NAM based on clinical presentation, imaging, and biopsy results.
- Positive anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibody titers confirmed the diagnosis.
- Treatment with corticosteroids and intravenous immunoglobulin led to significant clinical improvement.
Implications:
- This case underscores the importance of a comprehensive differential diagnosis in suspected immune-mediated myopathies.
- The presence of a heliotrope rash can be misleading and should not solely determine the diagnosis.
- Thorough investigation is critical to differentiate between conditions like DM and NAM, ensuring appropriate therapeutic strategies.

