ICD-11 classes
09 Diseases of the visual system
Strabismus or ocular motility disorders
Diseases of neuromuscular junction or muscle
Primary disorders of muscles
4A41 — Idiopathic inflammatory myopathy
4A41.0 — Dermatomyositis

ICD-11 4A41.0 — Dermatomyositis

Dermatomyositis is an inflammatory myopathy, showing progressive, symmetrical muscle weakness, low muscle endurance, and cutaneous manifestations such as Gottron’s papules, heliotrope rash, shawl sign, V-sign and mechanic’s hand. Internal organ manifestations such as interstitial pneumonia (pneumonitis) and myocarditis sometimes develop. The skin rash may precede the muscle symptoms and may be the only clinical sign of dermatomyositis in some patients (clinically, amyopathic dermatomyositis).

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 4A41.0 contains 3 clarifying diagnoses:

  1. 4A41.00 — Adult dermatomyositis
  2. 4A41.01 — Juvenile dermatomyositis
  3. 4A41.0Z — Dermatomyositis, unspecified

The diagnosis is coded elsewhere:

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