4A41.00

ICD-11 MMS 2026-01

Adult dermatomyositis

Description

Adult dermatomyositis is a systemic inflammatory disorder affecting the skeletal muscles, the skin, and other organs. It is characterised by symmetric proximal muscle weakness, increased serum muscle enzymes, myopathic changes upon electromyography, typical histological findings on muscle biopsy, and typical dermatologic manifestations such as heliotrope rash or Gottron’s papules.

Index terms

  • Adult dermatomyositis
  • Amyopathic dermatomyositis
  • Hypomyopathic dermatomyositis
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  • Adult dermatomyositis with calcinosis
  • Paraneoplastic dermatomyositis
  • Dermatomyositis in neoplastic disease of uncertain behaviour
  • Dermatomyositis in neoplastic disease of unknown behaviour
  • Dermatopolymyositis in neoplastic disease of uncertain behaviour
  • Dermatopolymyositis in neoplastic disease of unknown behaviour
  • Anti-synthetase syndrome with dermatomyositis
  • Adult dermatomyositis with overlap to nonorgan specific systemic autoimmune disorders
  • Wagner-Unverricht syndrome

Excludes

  • http://id.who.int/icd/entity/1425591047 – Myasthenia gravis or certain specified neuromuscular junction disorders

Exclusions from above levels

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

Sibling codes

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

Foundation entity

Adult dermatomyositis – http://id.who.int/icd/entity/544509908

Part of: 4A41.0: Dermatomyositis › 4A41: Idiopathic inflammatory myopathy › 04: Diseases of the immune system