Epidermolysis Bullosa
Pronunciation: ep-ih-der-MOL-ih-sis buh-LOH-suh
Also written as: EB — Epidermolysis Bullosa
A group of rare inherited disorders characterised by extreme fragility of skin and mucous membranes, causing blisters to form in response to minor trauma, with significant oral manifestations.
Full Definition
Epidermolysis bullosa (EB) encompasses a heterogeneous group of genodermatoses caused by mutations in genes encoding structural proteins of the skin and mucosal basement membrane zone. Oral manifestations include vesicle and bulla formation, gingival bleeding, microstomia, ankyloglossia, enamel hypoplasia, and rampant caries. Editors must note the correct spelling: 'epidermolysis bullosa', not 'epidermolysis bullous'. The abbreviation EB should be expanded on first use. Subtypes—EB simplex, junctional EB, dystrophic EB, and Kindler syndrome—must be specified precisely as they carry different prognoses and management implications.
Usage
Usage note: The adjective 'bullosa' does not inflect to 'bullous' in this compound noun. Distinguish subtype precisely (simplex, junctional, dystrophic) at first clinical mention.
In Context
- "Children with dystrophic epidermolysis bullosa require specialized dental protocols because routine instrumentation risks mucosal blistering." — Pediatric oral medicine guideline
- "The author wrote 'epidermolysis bullous' throughout; this was corrected to 'epidermolysis bullosa' at proofreading." — Proofreading correction log