EC32

ICD-11 MMS 2026-01

Dystrophic epidermolysis bullosa

Description

Dystrophic (dermolytic) epidermolysis bullosa is due to defects in or absence of type VII collagen. As a result the anchoring fibrils which secure the epidermal basement membrane to the dermis are defective or absent. The severe recessive form causes extensive scarring and predisposes to aggressive squamous cell carcinoma.

Index terms

  • Dystrophic epidermolysis bullosa
  • Dystrophic EB - [epidermolysis bullosa]
  • Epidermolysis bullosa dystrophica
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  • Autosomal dominant dystrophic epidermolysis bullosa
  • Dominant dystrophic epidermolysis bullosa, generalised
  • DDEB, generalised - [dominant dystrophic epidermolysis bullosa, generalised]
  • DDEB-gen - [dominant dystrophic epidermolysis bullosa, generalised]
  • Dominant dystrophic epidermolysis bullosa, acral
  • Acral dystrophic epidermolysis bullosa
  • DDEB-ac - [dominant dystrophic epidermolysis bullosa, acral]
  • DDEB-acral - [dominant dystrophic epidermolysis bullosa, acral]
  • Dominant dystrophic epidermolysis bullosa, pretibial
  • DDEB-Pt - [dominant dystrophic epidermolysis bullosa, pretibial]
  • DDEB-pretibial - [dominant dystrophic epidermolysis bullosa, pretibial]
  • Dominant dystrophic epidermolysis bullosa pruriginosa
  • DDEB pruriginosa - [dominant dystrophic epidermolysis bullosa pruriginosa]
  • DDEB-Pr - [dominant dystrophic epidermolysis bullosa pruriginosa]
  • Dominant dystrophic epidermolysis bullosa, nails only
  • DDEB, nails only - [dominant dystrophic epidermolysis bullosa, nails only]
  • DDEB-na - [dominant dystrophic epidermolysis bullosa, nails only]
  • Dystrophic epidermolysis bullosa, nails only
  • Dominant bullous dermolysis of the newborn
  • DDEB-BDN - [dominant bullous dermolysis of the newborn]
  • Autosomal recessive dystrophic epidermolysis bullosa
  • Recessive dystrophic epidermolysis bullosa, generalised severe
  • RDEB Hallopeau-Siemens
  • RDEB-GS - [recessive dystrophic epidermolysis bullosa, generalised severe]
  • RDEB-gen sev - [recessive dystrophic epidermolysis bullosa, generalised severe]
  • Recessive dystrophic epidermolysis bullosa, generalised intermediate
  • RDEB-O - [recessive dystrophic epidermolysis bullosa, generalised other]
  • RDEB-gen intermed - [recessive dystrophic epidermolysis, generalised intermediate]
  • Recessive dystrophic epidermolysis bullosa inversa
  • RDEB - [recessive dystrophic epidermolysis bullosa] inversa
  • RDEB-I - [recessive dystrophic epidermolysis bullosa inversa]
  • RDEB-inv - [recessive dystrophic epidermolysis bullosa inversa]
  • Recessive dystrophic epidermolysis bullosa, pretibial
  • RDEB - [recessive dystrophic epidermolysis bullosa] pretibial
  • RDEB-Pt - [recessive dystrophic epidermolysis bullosa, pretibial]
  • Recessive dystrophic epidermolysis bullosa pruriginosa
  • RDEB - [recessive dystrophic epidermolysis bullosa] pruriginosa
  • RDEB-Pr - [recessive dystrophic epidermolysis bullosa pruriginosa]
  • Recessive dystrophic epidermolysis bullosa centripetalis
  • RDEB - [recessive dystrophic epidermolysis bullosa centripetalis] centripetalis
  • RDEB-Ce - [recessive dystrophic epidermolysis bullosa centripetalis]
  • Recessive bullous dermolysis of the newborn
  • Bullous dermolysis of the newborn (recessive)
  • RDEB-BDN - [recessive bullous dermolysis of the newborn]
  • Recessive transient bullous dermolysis of the newborn

Exclusions from above levels

  • EB43 – Epidermolysis bullosa acquisita

Sibling codes

  • EC30 – Epidermolysis bullosa simplex
  • EC31 – Junctional epidermolysis bullosa
  • EC33 – Syndromic epidermolysis bullosa
  • EC3Z – Epidermolysis bullosa

Foundation entity

Dystrophic epidermolysis bullosa – http://id.who.int/icd/entity/1060981106

Part of: 14: Diseases of the skin