| • | cleavage beneath the lamina densa |
| • | characterized by scarring, nail changes, and milia |
| • | recessive dystrophic EB – complete absence of anchoring fibrils |
| • | dominant dystrophic EB – reduced number and/or abnormal in appearance |
autosomal recessive (Hallopeau-Siemens)
| • | etiology = type VII collagen (anchoring fibrils) (same as epidermolysis bullosa acquisita) |
| • | large bullae heal with atrophic scars and milia: pseudo-webbing = “mitten hands” |
| • | mucous membranes often involved (may lead to esophageal stricture formation; any epithelial lined organ) |
| • | corneal erosions, cicatricial alopecia |
autosomal dominant (Cockayne-Touraine)
|