Types of Dermatomyositis

Dermatomyositis is classified into different subtypes based on the extent of skin or muscle involvement:

Adermatopathic dermatomyositis (Dermatomyositis sine dermatitis)

•  Muscle weakness and histopathological signs similar to dermatomyositis, without the cutaneous features3,5
•  Transient or poorly recognised rash2 
•  Many of these patients are likely misclassified and instead may be cases of overlap myositis5

Myopathic dermatomyositis

•  Characteristic skin rash together with muscle weakness1-3

Amyopathic dermatomyositis (Dermatomyositis sine myositis)

•  Cutaneous features without clinical muscle weakness for ≥ 6 months4
•  On biopsy, subclinical muscular involvement often detected2  
•  Accounts for 5–20% of patients with dermatomyositis4,5

Juvenile dermatomyositis occurs in patients < 18 years old at onset3

Juvenile dermatomyositis is the most common inflammatory myopathy of childhood. Juvenile Dermatomyositis is characterized by the presence of the typical rash and proximal muscle weakness. Compared to dermatomyositis in adults, pediatric dermatomyositis patients show greatest weakness in hip flexors, extensors, and adductors, as well as neck flexors and shoulder abductors. Characteristic dermatologic features of juvenile dermatomyositis involve the heliotrope rash, Gottron’s papules, and nail fold changes. Calcinosis is a common finding (up to 40% of patients) in juvenile dermatomyositis.6

managedermatomyositis.com, breakout-on-hands-gottron-signs
Gottron signs on hands (courtesy of Prof. Patrick Cherin)

Dermatomyositis affects both adults and children1, with subtypes varying in the extent of skin and muscle involvement.1-5

Image shows Gottron's signs on hands. Gottron’s signs are skin changes that may be seen in people living with dermatomyositis. These signs are typically observed on the knuckles and fingers and may include redness of the skin (Erythema).2

References

  1. Goyal, N. A. (2019). Immune-Mediated Myopathies. CONTINUUM: Lifelong Learning in Neurology, 25(6), 1564–1585. https://doi.org/10.1212/con.0000000000000789
  2. Dalakas, M. C., & Hohlfeld, R. (2003). Polymyositis and dermatomyositis. The Lancet, 362(9388), 971–982. https://doi.org/10.1016/s0140-6736(03)14368-1
  3. Malik, A., Hayat, G., Kalia, J. S., & Guzman, M. A. (2016). Idiopathic Inflammatory Myopathies: Clinical Approach and Management. Frontiers in Neurology, 7, 64.  https://doi.org/10.3389/fneur.2016.00064
  4. Callander, J., Robson, Y., Ingram, J., & Piguet, V. (2016). Treatment of clinically amyopathic dermatomyositis in adults: a systematic review. British Journal of Dermatology, 179(6), 1248–1255. https://doi.org/10.1111/bjd.14726
  5. Schmidt, J. (2018). Current Classification and Management of Inflammatory Myopathies. Journal of Neuromuscular Diseases, 5(2), 109–129. https://doi.org/10.3233/jnd-180308
  6. Swafford, C., and Roach, E.S. (2020). Juvenile Dermatomyositis and the Inflammatory Myopathies. Semin. Neurol. 40, 342-348. https://doi.org/10.1055/s-0040-1705120

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