Epidermolysis Bullosa

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•	AKA mechanobullous disorders

•	“epidermolysis” is a misnomer because the dystrophic forms have “dermolysis” (with a normal epidermis)

EB simplex: (intraepidermal split; autosomal dominant)

•	localized (hand and foot): Weber-Cockayne

•	generalized: Köbner

Junctional EB: lamina lucida split (with atrophy; autosomal recessive)

•	Lethal (gravis, Herlitz)

•	Nonlethal (mitis, non-Herlitz, GABEB)

Dystrophic EB: sub-lamina densa split (with scarring)

•	autosomal recessive (most severe)

•	autosomal dominant

disease:

EBS

junctional EB

•	lethal EB (Herlitz)

•	EB with pyloric atresia

•

GABEB

•	EB with muscular dystrophy

dystrophic EB

mutation:

keratin 5,14

laminin 5

•

laminin 5

•	α6β4 integrin

•	BP180 antigen

•

plectin

type VII collagen