title: Squamous cell carcinoma in junctional and dystrophic epidermolysis bullosa.
abstract: We report here on three patients suffering from recessive dystrophic epidermolysis bullosa and one suffering from generalized atrophic benign epidermolysis bullosa, all of whom developed cutaneous squamous cell carcinoma. Our observations and a review of the literature suggest that squamous cell carcinoma in generalized atrophic benign epidermolysis bullosa is very infrequent and has a better outcome compared to skin cancer in recessive dystrophic epidermolysis bullosa. These differences could be explained by the distinct pathophysiology and clinical course of each of these variants of epidermolysis bullosa. In contrast to UV-induced skin cancer, the tumours in epidermolysis bullosa develop on distal extremities at sites of chronic wound healing. The cases reported here underline the exceptional importance of early histopathological assessment of suspicious skin lesions in patients with epidermolysis bullosa.
authors.affiliation: Department of Dermatology and Venereology, University of Innsbruck, Austria. florian.weber@uibk.ac.at
authors.forename: F
authors.initials: F
authors.lastname: Weber
authors.forename: J W
authors.initials: JW
authors.lastname: Bauer
authors.forename: N
authors.initials: N
authors.lastname: Sepp
authors.forename: W
authors.initials: W
authors.lastname: Högler
authors.forename: W
authors.initials: W
authors.lastname: Salmhofer
authors.forename: H
authors.initials: H
authors.lastname: Hintner
authors.forename: P
authors.initials: P
authors.lastname: Fritsch
mesh_terms: D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000150:complications* / Q000473:pathology; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications* / Q000473:pathology; D016109:Epidermolysis Bullosa, Junctional / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000150:complications* / Q000473:pathology