Oral Alterations in Heritable Epidermolysis Bullosa: A Clinical Study and Literature Review

Table 1

Principal oral alterations in inherited forms of EB.

Major EB subtype	Variants	Soft tissue alteration	Hard tissue alteration
EBS	Localized and generalized	Possible blistering and ulceration, usually localized and without scarring	—
JEB	Intermediate	Possible blistering and ulceration without scarring	Moderate enamel hypoplasia and risk of rampant caries
	Severe	Possible blistering and ulceration without scarring, perioral granulation tissue, and microstomia	Severe enamel hypoplasia and risk of rampant caries
DEB	Dominant	Tissue fragility, usually without blistering	—
	Recessive	Diffuse ulcerations and scarring: ankyloglossia, microstomia, loss of palatal rugae and lingual papillae, and vestibule obliteration	No enamel alterations but high risk of rampant caries due to soft diet, slow eating and worse oral hygiene
KEB	—	Sever blistering during infancy which diminishes with age	Risk for severe early-onset periodontal disease

Rare variants.