EC31

ICD-11 MMS 2026-01

Junctional epidermolysis bullosa

Description

Junctional (lucidolytic) epidermolysis bullosa is due to defects at the level of the lamina lucida of the epidermal basement membrane. The generalised severe form (Herlitz) is associated with widespread mucosal involvement of internal organs and has a high mortality in infancy.

Index terms

  • Junctional epidermolysis bullosa
  • JEB - [junctional epidermolysis bullosa]
  • Junctional EB - [epidermolysis bullosa]
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  • Lucidolytic epidermolysis bullosa
  • Junctional epidermolysis bullosa, generalised types
  • Junctional epidermolysis bullosa, generalised severe
  • Epidermolysis bullosa letalis
  • Herlitz junctional epidermolysis bullosa gravis
  • Herlitz syndrome
  • JEB-H - [junctional epidermolysis bullosa, Herlitz]
  • JEB-gen sev - [junctional epidermolysis bullosa, generalised severe]
  • Junctional epidermolysis bullosa, generalised intermediate type
  • GABEB - [generalised atrophic benign epidermolysis bullosa]
  • JEB - [junctional epidermolysis bullosa] non-Herlitz, generalised
  • JEB-gen intermed - [junctional epidermolysis bullosa]
  • JEB-nH gen - [junctional epidermolysis bullosa, non-Herlitz, generalised]
  • generalised atrophic benign epidermolysis bullosa
  • Junctional epidermolysis bullosa with pyloric atresia
  • JEB - [junctional epidermolysis bullosa] with pyloric atresia
  • JEB-PA - [junctional epidermolysis bullosa with pyloric atresia]
  • Junctional epidermolysis bullosa, late onset
  • Epidermolysis bullosa progressiva
  • JEB - [junctional epidermolysis bullosa] late onset
  • JEB-LO - [junctional epidermolysis bullosa late onset]
  • Junctional epidermolysis bullosa with respiratory and renal involvement
  • Junctional epidermolysis bullosa, localised types
  • Junctional epidermolysis bullosa, localised
  • JEB - [junctional epidermolysis bullosa] non-Herlitz, localised
  • JEB-loc - [junctional epidermolysis bullosa, localised
  • JEB-nH loc - [junctional epidermolysis bullosa, non-Herlitz, localised]
  • Junctional epidermolysis bullosa inversa
  • JEB - [junctional epidermolysis bullosa] inversa
  • JEB-inv - [junctional epidermolysis bullosa inversa]
  • Junctional epidermolysis bullosa, LOC
  • JEB-LOC - [junctional epidermolysis bullosa laryngo-onycho-cutaneous] syndrome
  • LOC - [laryngo-onycho-cutaneous] syndrome
  • LOGIC
  • Laryngo-onycho-cutaneous syndrome
  • Shabbir syndrome

Exclusions from above levels

  • EB43 – Epidermolysis bullosa acquisita

Sibling codes

  • EC30 – Epidermolysis bullosa simplex
  • EC32 – Dystrophic epidermolysis bullosa
  • EC33 – Syndromic epidermolysis bullosa
  • EC3Z – Epidermolysis bullosa

Foundation entity

Junctional epidermolysis bullosa – http://id.who.int/icd/entity/1501260457

Part of: 14: Diseases of the skin