EB simplex (EBS) |
EBS, localized | Basal layer | Split may spread to suprabasilar layer |
EBS, DM | Basal layer in subnuclear cytoplasm | Dense, circumscribed clumps of keratin filaments (most commonly observed |
EBS-MD | Predominantly in basal layer, above level of HD attachment plaque | Reduced integration of keratin filaments with HD |
EBS-AR | Basal keratinocytes | Absent or reduced keratin filaments within basal keratinocytes |
EBSS | Split usually at interface between granular and cornified cell layers | — |
EBS, lethal acantholytic | Suprabasal cleavage and acantholysis | Perinuclear retraction of keratin filaments |
EBS, plakophilin-1 deficiency | Midepidermal cell-cell separation | Diminutive suprabasal desmosomes; perinuclear retraction of keratin filaments |
EBS-PA | Lower basal layer, above level of HD plaque | Reduced integration of keratin filaments with HD |
Junctional EB (JEB) |
JEB-H | Lamina lucida | Markedly reduced or absent HD; absent SBDP |
JEB-nH | Lamina lucida | HDs may be normal or reduced in size and number |
JEB-PA | Lamina lucida | Small HD plaques often with attenuated SBDP |
Dominant dystrophic EB (DDEB) |
DDEB, generalized | Sublamina densa | Normal or decreased numbers of AFs |
DDEB-BDN | Sublamina densa | Electron-dense stellate bodies within basal layer; reduced AFs |
Recessive dystrophic EB (RDEB) |
RDEB, severe generalized | Sublamina densa | Absent or rudimentary AFs |
RDEB, generalized other | Sublamina densa | Reduced or rudimentary-appearing AFs |
RDEB-BDN | Sublamina densa | Electron-dense stellate bodies within basal layer; reduced AFs |