Conflict of interest: none declared.
A systematic review of randomized controlled trials of treatments for inherited forms of epidermolysis bullosa
Article first published online: 26 SEP 2008
© 2008 The Author(s). Journal compilation © 2008 Blackwell Publishing Ltd
Clinical and Experimental Dermatology
Volume 34, Issue 1, pages 20–25, January 2009
How to Cite
Langan, S. M. and Williams, H. C. (2009), A systematic review of randomized controlled trials of treatments for inherited forms of epidermolysis bullosa. Clinical and Experimental Dermatology, 34: 20–25. doi: 10.1111/j.1365-2230.2008.02789.x
- Issue published online: 8 DEC 2008
- Article first published online: 26 SEP 2008
- Accepted for publication 21 December 2007
Background. Many interventions have been described for inherited epidermolysis bullosa (EB), but it is unclear which are beneficial.
Aims. A systematic review of randomized controlled trials (RCTs) was performed to inform practice and highlight research gaps.
Methods. The Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE and the Cochrane Skin Group specialist library, from inception until 1 April 2007, were searched. Primary outcomes were healing of lesions or prevention of new lesions. Trials were assessed for quality of reporting and data were extracted.
Results. Five randomized double-blind placebo-controlled crossover studies were identified (n = 102). Two studies assessed oral tetracyclines in EB simplex (EBS). In one study (n = 12), 4/6 patients improved and 2/6 deteriorated on a dose of 1500 mg of tetracycline daily; only two patients completed the study. In the second study (n = 21), 6/18 and 7/18 improved on oxytetracycline 1 g and placebo, respectively. Two RCTs assessed topical interventions for EBS: aluminium chloride hexahydrate solution 20% (n = 23) and bufexamac cream 5% (n = 8). Neither showed a benefit over placebo. One RCT of 36 patients with recessive dystrophic EB compared phenytoin with placebo and failed to show any difference in mean lesion counts (difference = 0, 95% CI −11 to 4).
Conclusions. There is no reliable trial evidence for interventions in inherited EB. In future, it may be that gene treatment becomes the best treatment approach for these diseases.