| • | blister formation within the lamina lucida (laminin 5 defect) |
Lethal junctional eb: (Herlitz)
| • | generalized blistering and erosions at birth; most severe form of JEB |
| • | pathognomonic clinical finding = thickened areas of exuberant granulation tissue (especially around mouth and nostrils, posterior neck) |
| • | nails are involved and eventually shed |
| • | oral mucosa often involved; any epithelial lined organ may be affected (mouth, esophagus, small intestine) |
| • | eroded areas slow to heal; atrophic scars but no milia |
| • | many infants die early with overwhelming infection |
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