Mariana Armada, Dr. Adela Stepanska, Dr. Renata Gaillyova, Dr. Sylvia Stepanska, Mr. John Dart, Mr. Scott O Sullivan, Dr. David Peñarrocha, Prof. Dr. Tim Wright, Dr. Marie Callen, Dr. Carol Mason, Prof. Dr. Stephen Porter, Dr. Nina this website Skogedal, Dr. Kari Storhaug, Dr. Reinhard Schilke, Prof. Dr. Marco Cornejo,

Dr. Anne W Lucky, Lesley Haynes, Lynne Hubbard, Isabel López and Christian Fingerhuth for their contribution to these guidelines, as it has been detailed on chapter 6. This work was funded by a grant from DEBRA UK. None of the authors declared conflict of interest. Abbreviations EB Epidermolysis bullosa EBS EB simplex JEB Junctional EB DEB Dystrophic EB RDEB Recessive DEB DDEB Dominant DEB RDEB, sev gen Severe generalized RDEB SCC Squamous cell carcinoma The frequency and severity of the oral manifestations of EB vary with the type of disease; however, in general, the oral mucosal lesions of EB comprise vesiculobullous lesions that vary from small, discrete vesicles to large bullae. These lesions can be distributed on all of the mucosal surfaces. Differences exist with regard to the prevalence and severity of oral involvement SCH727965 solubility dmso among the different

EB types, patients with the generalized RDEB being the most severely affected19,28. The hard tissues also present different involvement depending on the form of EB. Patients with JEB present with generalized enamel hypoplasia, and individuals with RDEB and JEB have significantly more caries when compared with other EB types or unaffected controls, whereas patients with EBS and DDEB do not have an increased caries risk19. Although the most recent classification58 considers two major subtypes and 12 minor subtypes of EBS, most of the literature on the oral aspects of EBS precedes this classifications system;

hence, the following text will consider the oral manifestations of EBS as a group and will only reflect on the subtype when available. Oral ulcers.  Oral mucosal ulceration was described in 2% of patients with EBS in an early report. A more recent case series reported greater involvement, although oral mucosal involvement was not always determined by direct clinical examination but by a history of oral ulceration28. 40.3% of the group of 124 clonidine patients with EBS had oral ulcers with 58.6% of those with generalized and 34.7% with localized EB. Oral mucosal involvement was reported to be more common during the perinatal period, but in some patients, it persisted during early childhood or even later (Image 13)28. EBS, localized (EBS-loc) (previously termed EBS Weber-Cockayne) There is some dispute as to the frequency of oral mucosal lesions in EBS-loc. Whereas Sedano59 reported this subtype does not give rise to oral mucosal lesions, Wright28 reported that 34.7% (33/95) of the patient with localized EBS had a history of or presence of oral mucosal blisters at examination.