4A41.0

ICD-11 MMS 2026-01

Dermatomyositis

Description

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).

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Child codes

  • 4A41.00 – Adult dermatomyositis
  • 4A41.01 – Juvenile dermatomyositis
  • 4A41.0Z – Dermatomyositis, unspecified

Sibling codes

  • 4A41.1 – Polymyositis
  • 4A41.2 – Inclusion body myopathy
  • 4A41.Y – Other specified idiopathic inflammatory myopathy
  • 4A41.Z – Idiopathic inflammatory myopathy, unspecified

Foundation entity

Dermatomyositis – http://id.who.int/icd/entity/739030149

Part of: 4A41: Idiopathic inflammatory myopathy › 04: Diseases of the immune system