Thanks to Dr. Erica Tate for a great presentation of Dermatomyositis!
Learning Points:
- Diagnostic criteria: symmetric proximal muscle weakness, elevation of muscle enzyme levels, abnormal EMG results, muscle biopsy abnormalities and typical skin rash
- Pathogenesis: complement mediated vasculopathy of the small vessels in muscle tissue resulting in ischemia and vessel damage
- Clinical presentation: classically presents with heliotrope rash, gottron’s papules and other dermatologic manifestations prior to onset of proximal muscle weakness. Can also affect cardiovascular, pulmonary and GI systems
- Treatment: gold standard is initiation of corticosteroids followed by replacement with steroid-sparing agents such as Methotrexate, Mycophenolate mofetil and Azathioprine. In refractory cases, other treatment options include IVIG, Cyclosporin and Rituximab
Enjoy this great article on Dermatomyositis for further learning!