Table 1 Major epidermolysis bullosa complications affecting the skin, eye and ENT area
From: Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa
Tissue/organ/system | Major complications | EB type/subtype* |
|---|---|---|
Skin | Fluid loss | Lethal acantholytic EB, JEB-H, EBS-PA, JEB-PA |
Chronic/infected wounds | RDEB-SG, RDEB-I, RDEB-O, JEB-H, JEB-nH, EBS-AR, DEB-Pr, DDEB-G, EBS-DM | |
Exuberant granulation tissue | LOC, JEB-H, JEB-nH | |
Atrophic scars, post-inflammatory pigmentary changes | DEB, JEB, EBS | |
Poikiloderma/diffuse skin atrophy | KS | |
Excessive/hypertrophic scarring | RDEB-SG, RDEB-I, DEB-Pt, DEB-Pr, RDEB-O | |
Albopapuloid lesions | DEB | |
Milia | DEB, JEB, EBS, KS | |
Palmoplantar keratoderma | EBS, JEB-nH, KS | |
Aplasia cutis congenita | EBS-PA, JEB-PA, DEB, other JEB and EBS subtypes | |
EB nevi | JEB, DEB, EBS | |
Basal cell carcinomas | EBS-DM | |
Squamous cell carcinomas | RDEB-SG, RDEB-O, KS, JEB-nH, RDEB-I | |
Skin adnexa | Onychodystrophy, nail shedding or loss | DEB, JEB, EBS-MD, EBS-DM, EBS-PD, EBS-AR, EBS-O, KS |
Scarring alopecia | JEB-nH, RDEB-SG, JEB-H, JEB-PA, RDEB-O | |
Alopecia universalis | Lethal acantholytic EB | |
Hypotrichosis | EBS-PD | |
Oral cavity | Microstomia, ankyloglossia, obliteration of the oral vestibules | RDEB-SG, RDEB-I, RDEB-O |
Enamel hypoplasia | JEB, EBS-MD | |
Multiple caries and tooth decay | DEB, JEB | |
Periodontitis | KS | |
External eye | Corneal erosions | RDEB-SG, JEB-H, RDEB-O, RDEB-I, JEB-nH, EBS-DM, KS |
Blepharitis, corneal scarring and/or pannus formation | RDEB-SG, RDEB-I, JEB-H, RDEB-O, JEB-nH | |
Symblepharon | LOC, RDEB-SG, RDEB-I, JEB-H, JEB-nH | |
Ectropion/exposure keratitis | JEB-H, RDEB-SG, KS | |
Diminished vision/blindness | RDEB-SG | |
Conjunctival granulation tissue | LOC | |
External ear | External auditory canal narrowing/conductive hearing loss | RDEB-I |
Nose | Nare narrowing (granulation tissue) | JEB-H, JEB-nH, LOC |