EB Type and Subtype

Sub-lamina densa ultrastructural findings

Antigenic alterations

Dominant dystrophic EB (DDEB)

DDEB, generalized

Normal or decreased numbers of anchoring fibrils

DDEB-BDN

Electron-dense stellate bodies within basal layer; reduced anchoring fibrils

Type VII collagen:  granular staining within basal and suprabasal keratinocytes; absent or markedly reduced staining along DEJ (only during period of active blistering)

Recessive dytrophic EB (RDEB)

RDEB, severe generalized

Absent or rudimentary anchoring fibrils

Type VII collagen absent or markedly reduced

RDEB, generalized other

Reduced or rudimentary-appearing anchoring fibrils

Type VII collagen reduced

RDEB-BDN

Electron-dense stellate bodies within basal layer; reduced anchoring fibrils