BACKGROUND: Inherited epidermolysis bullosa is a rare disease characterised by mechanical fragility of the skin when under insignificant stress. The main consequences of epidermolysis bullosa, mainly the dystrophic type, despite pseudosyndactyly, are joint contractures and deformities in hands and feet. In this study, we describe our experience treating patients suffering from epidermolysis bullosa, as far as feet deformities are concerned. METHODS: This is a retrospective analysis of a consecutive series of patients presenting feet deformities related to epidermolysis bullosa. Extension contractures of the toes, equinus and cavus deformities were treated with soft tissues surgery. RESULTS: Thirteen surgical procedures were done in six patients with feet deformities caused by epidermolysis bullosa. Of the feet operated 85.7 % extension contracture of the toes was asymptomatic at follow-up. However, 42.9 % developed hammertoe deformities. There were no recurrence or complications for other deformities. Subjectively, all patients declared themselves very satisfied with the results. CONCLUSION: Foot deformities must be treated as early as possible, due to progressive disability for walking and pain symptoms. We considered that, despite long term complications, treatment was adequate and we recommend it. Level of Evidence Level IV.
BACKGROUND: Inherited epidermolysis bullosa is a rare disease characterised by mechanical fragility of the skin when under insignificant stress. The main consequences of epidermolysis bullosa, mainly the dystrophic type, despite pseudosyndactyly, are joint contractures and deformities in hands and feet. In this study, we describe our experience treating patients suffering from epidermolysis bullosa, as far as feet deformities are concerned. METHODS: This is a retrospective analysis of a consecutive series of patients presenting feet deformities related to epidermolysis bullosa. Extension contractures of the toes, equinus and cavus deformities were treated with soft tissues surgery. RESULTS: Thirteen surgical procedures were done in six patients with feet deformities caused by epidermolysis bullosa. Of the feet operated 85.7 % extension contracture of the toes was asymptomatic at follow-up. However, 42.9 % developed hammertoe deformities. There were no recurrence or complications for other deformities. Subjectively, all patients declared themselves very satisfied with the results. CONCLUSION:Foot deformities must be treated as early as possible, due to progressive disability for walking and pain symptoms. We considered that, despite long term complications, treatment was adequate and we recommend it. Level of Evidence Level IV.
Authors: Jo-David Fine; Robin A J Eady; Eugene A Bauer; Johann W Bauer; Leena Bruckner-Tuderman; Adrian Heagerty; Helmut Hintner; Alain Hovnanian; Marcel F Jonkman; Irene Leigh; John A McGrath; Jemima E Mellerio; Dedee F Murrell; Hiroshi Shimizu; Jouni Uitto; Anders Vahlquist; David Woodley; Giovanna Zambruno Journal: J Am Acad Dermatol Date: 2008-04-18 Impact factor: 11.527
Authors: M T Khan; M O'Sullivan; B Faitli; J E Mellerio; R Fawkes; M Wood; L D Hubbard; A G Harris; L Iacobaccio; T Vlahovic; L James; L Brains; M Fitzpatrick; K Mayre-Chilton Journal: Br J Dermatol Date: 2019-10-23 Impact factor: 9.302