Bardhan A, Bruckner-Tuderman L, Chapple ILC, Fine JD, Harper N, Has C, Magin TM, Marinkovich MP, Marshall JF, McGrath JA, Mellerio JE, Polson R, Heagerty AH. Epidermolysis bullosa. Nat Rev Dis Primers. 2020 Sep 24;6(1):78.

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Extracutaneous manifestations in EB. Extracutaneous manifestations are more frequent in severe forms of epidermolysis bullosa (EB) than in milder forms. However, some specific subtypes may present with specific disease complications. For example, junctional EB can present with interstitial lung disease and nephrotic syndrome and EB simplex can occur with pyloric atresia. Microstomia, ankyloglossia and pseudosyndactyly are common in severe recessive dystrophic EB, reflecting the degree and depth of repeated blistering, inflammation, scarring and fibrosis that occurs. a Mainly severe recessive dystrophic EB. b EB simplex with plectin mutations. c Severe recessive dystrophic EB only.