Table 2.

Junctional Epidermolysis Bullosa Nomenclature

Current Nomenclature2008 Nomenclature2013 "Onion Skin"
Nomenclature		Other Specific JEB Designations
Used in the Past
JEB SubtypeAbbreviation
GeneralizedJEB, generalized severeJEB-gen sevHerlitz JEB
(H-JEB)		JEB generalized severe, laminin-332 absent, LAMA3, LAMB3, or LAMC2 pathogenic variants (specify type)
  • Epidermolysis bullosa letalis
  • Epidermolysis bullosa junctional Herlitz-Pearson
  • Junctional epidermolysis bullosa mitis
JEB, generalized intermediateJEB-gen intermedNon-Herlitz JEB
(NH-JEB)		JEB generalized intermediate, laminin-332 or collagen XVII reduced staining, LAMA3, LAMB3, LAMC2, or COL17A1 pathogenic (specify type)
  • Epidermolysis bullosa, generalized atrophic benign (GABEB)
  • Epidermolysis bullosa junctionalis, disentis type
  • Epidermolysis bullosa junctionalis, progressive
  • Epidermolysis bullosa junctionalis, severe non-lethal
JEB with pyloric atresiaJEB-PA
JEB-late onsetJEB-LO
JEB with respiratory and renal involvementJEB-RR
LocalizedJEB, localizedJEB-loc
JEB, inversaJEB-inv; JEB-I
JEB-LOC syndrome

Adapted from Fine et al [2014]

JEB = junctional epidermolysis bullosa

From: Junctional Epidermolysis Bullosa

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