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Table 3 Transmission electron microscopy findings among selected EB subtypes

From: Inherited epidermolysis bullosa

EB type Major EB subtype Level of cleavage Associated ultrastructural findings
EB simplex (suprabasal) EB simplex superficialis subcorneal ---
  lethal acantholytic EB suprabasal acantholysis; perinuclear retraction of keratin filaments
  EBS, plakophilin deficiency mid-epidermis perinuclear retraction of keratin filaments; small suprabasal desmosomes
EBS (basal) EBS, localized basal keratinocyte ---
  EBS, Dowling-Meara basal keratinocyte clumped keratin filaments
  EBS, generalized other basal keratinocyte ---
  EBS, autosomal recessive basal keratinocyte absent or reduced keratin filaments within basal keratinocytes
Junctional EB JEB, Herlitz intra-lamina lucida markedly reduced or absent hemidesmosomes; absent subbasal dense plates; absent anchoring filaments
  JEB, non-Herlitz intra-lamina lucida hemidesmosomes may be normal or reduced in size and number
  JEB with pyloric atresia intra-lamina lucida small hemidesmosome plaques with attenuated subbasal dense plates
Dominant dystrophic EB DDEB, generalized sub-lamina densa normal or reduced numbers of anchoring fibrils
  DDEB, bullous dermolysis of the newborn sub-lamina densa electron-dense stellate shaped bodies within basal keratinocytes; reduced numbers of anchoring fibrils
Recessive dystrophic EB RDEB, severe generalized sub-lamina densa absent or rudimentary appearing anchoring fibrils
  RDEB, generalized other (generalized mitis) sub-lamina densa reduced or rudimentary appearing anchoring fibrils