EC30

ICD-11 MMS 2026-01

Epidermolysis bullosa simplex

Description

Epidermolysis bullosa simplex is the name given to a heterogeneous group of genetically-determined defects in epidermal cell-cell adhesion. These give rise to blistering in response to frictional and shearing stresses.

Index terms

  • Epidermolysis bullosa simplex
  • Epidermolytic epidermolysis bullosa
  • Suprabasal epidermolysis bullosa simplex
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  • Suprabasal EBS
  • Acantholytic epidermolysis bullosa
  • EBS-acanth
  • LAEB
  • LAEB - [lethal acantholytic epidermolysis bullosa] due to desmoplakin deficiency (MIM 609638)
  • Lethal acantholytic epidermolysis bullosa
  • Epidermolysis bullosa simplex superficialis
  • EBS - [epidermolysis bullosa simplex] superficialis
  • EBSS - [epidermolysis bullosa simplex superficialis]
  • Ectodermal dysplasia – skin fragility syndrome
  • EBS-plakophilin
  • ED – SF - [Ectodermal dysplasia – skin fragility] syndrome (MIM 604536)
  • Plakophilin 1 deficiency
  • Skin fragility - ectodermal dysplasia syndrome
  • Skin fragility - plakoglobin deficiency
  • EBS-plakoglobin
  • Basal epidermolysis bullosa simplex
  • Basal EBS - [basal epidermolysis bullosa simplex]
  • Autosomal dominant basal epidermolysis bullosa simplex
  • Epidermolysis bullosa simplex, localised
  • Epidermolysis bullosa simplex, Weber-Cockayne
  • localised EBS- [epidermolysis bullosa simplex]
  • Epidermolysis bullosa simplex with mottled pigmentation
  • EBS - [epidermolysis bullosa simplex] with mottled pigmentation
  • EBS-MP - [epidermolysis bullosa simplex with mottled pigmentation]
  • Epidermolysis bullosa simplex, Ogna
  • EBS, Ogna - [epidermolysis bullosa simplex, Ogna]
  • EBS-Og - [epidermolysis bullosa simplex, Ogna]
  • Epidermolysis bullosa simplex, generalised severe
  • EBS - [epidermolysis bullosa simplex] Dowling-Meara
  • EBS-DM - [generalised epidermolysis bullosa simplex, Dowling-Meara]
  • EBS-gen sev - [epidermolysis bullosa simplex generalised type]
  • Epidermolysis bullosa herpetiformis
  • Epidermolysis bullosa simplex, generalised intermediate
  • EBS - [epidermolysis bullosa simplex] non-Dowling-Meara
  • EBS, gen-nDM - [generalised epidermolysis bullosa simplex, non-Dowling-Meara]
  • EBS-gen intermed
  • Autosomal recessive basal epidermolysis bullosa simplex
  • Epidermolysis bullosa simplex, migratory circinate
  • EBS - [epidermolysis bullosa simplex] migratory circinate
  • Epidermolysis bullosa simplex with muscular dystrophy
  • EBS - [epidermolysis bullosa simplex] with muscular dystrophy
  • EBS-MD - [epidermolysis bullosa simplex with muscular dystrophy]
  • Epidermolysis bullosa simplex with limb-girdle muscular dystrophy
  • Epidermolysis bullosa simplex with pyloric atresia
  • EBS - [epidermolysis bullosa simplex] with pyloric atresia
  • EBS-PA - [epidermolysis bullosa simplex with pyloric atresia]
  • Autosomal recessive epidermolysis bullosa simplex due to BP230 deficiency
  • EBS - [epidermolysis bullosa simplex] new subtype
  • EBS - [epidermolysis bullosa simplex] other specified autosomal recessive
  • EBS-AR BP230
  • Epidermolysis bullosa simplex, autosomal recessive due to exophilin 5 deficiency
  • EBS - [epidermolysis bullosa simplex] autosomal recessive
  • EBS-AR - [epidermolysis bullosa simplex, autosomal recessive]
  • EBS-AR exophilin 5
  • Epidermolysis bullosa simplex, autosomal recessive keratin 14 deficiency
  • EBS-AR K14 - [epidermolysis bullosa simplex - autosomal recessive keratin 14]

Exclusions from above levels

  • EB43 – Epidermolysis bullosa acquisita

Sibling codes

  • EC31 – Junctional epidermolysis bullosa
  • EC32 – Dystrophic epidermolysis bullosa
  • EC33 – Syndromic epidermolysis bullosa
  • EC3Z – Epidermolysis bullosa

Foundation entity

Epidermolysis bullosa simplex – http://id.who.int/icd/entity/1860717527

Part of: 14: Diseases of the skin