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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
Palmoplantar keratoderma EBS, JEB-nH, KS
Aplasia cutis congenita EBS-PA, JEB-PA, DEB, other JEB and EBS subtypes
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
  1. *The epidermolysis bullosa (EB) types and subtypes which present a higher frequency of a given complication are listed first.
  2. EBS, epidermolysis bullosa simplex; EBS-PA, EBS with pyloric atresia; EBS-DM, EBS, Dowling-Meara; EBS-MD, EBS with muscular dystrophy; EBS-AR, EBS, autosomal recessive; EBS-PD, plakophilin deficiency; EBS-O, EBS, generalized other; JEB, junctional epidermolysis bullosa; JEB-H, JEB, Herlitz; JEB-nH, JEB, non-Herlitz; JEB-PA, JEB with pyloric atresia; LOC, laryngo-onycho-cutaneous syndrome; DEB, dystrophic epidermolysis bullosa; DDEB, dominant DEB; DDEB-G, DDEB, generalized; RDEB-SG, recessive DEB, severe generalized; RDEB-O, recessive DEB, generalized other; RDEB-I, recessive DEB, inversa; DEB-Pr, DEB, pruriginosa; DEB-Pt, pretibial DEB; KS, Kindler syndrome.