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Junctional epidermolysis bullosa, generalized severe

Disease definition

Junctional epidermolysis bullosa, Herlitz-type is a severe subtype of junctional epidermolysis bullosa (JEB, see this term) characterized by blisters and extensive erosions, localized to the skin and mucous membranes.


  • Synonym(s):
    • Epidermolysis bullosa letalis
    • JEB, generalized severe
    • JEB-H
    • Junctional epidermolysis bullosa generalisata gravis
    • Junctional epidermolysis bullosa, Herlitz type
    • Junctional epidermolysis bullosa, Herlitz-Pearson type
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q81.1
  • OMIM: 226700
  • UMLS: C0079683
  • MeSH: -
  • GARD: 2153
  • MedDRA: -

Detailed information

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Additional information

Further information on this disease

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