Few patients have continuous blister formation on the oral mucous membranes. These blisters heal without scarring73. this website Dominant DEB (DDEB) There is no agreement about the extent of oral mucosal involvement in DDEB. One review stated that 20% of patients have oral mucosal bullae59, although a case series indicated that 71.1–89.6% of patients may have a history of or oral clinical features of oral mucosal blistering (Images 18 and 19)5,28. Of note, significant scarring, vestibular obliteration, and ankyloglossia do not seem to be long-term complications of oral mucosal ulceration/blisters28. Hard tissue involvement. Patients with DDEB do
not seem to be at increased risk of caries5,19. Recessive DEB (RDEB) RDEB inversa (RDEB-I) RDEB inversa subtype is an uncommon
form of EB. Patients present with mucosal blistering (especially sublingually), ankyloglossia, absence of tongue papillae and palatal rugae, partial obliteration of the vestibule, microstomia secondary to scarring, and mucosal milia5,74,75. Of note, oesophageal involvement and dysphagia affected 90% of one group of ten patients74. Hard tissue involvement. A significantly higher prevalence of caries (DMFS: 50.9) than the control group (DMFS: 12.8) was reported in a study of ten patients. Enamel abnormalities PI3K inhibitor have only been reported in one of 14 patients having a localized enamel defect of one tooth74. The following text includes all patients with both generalized forms of RDEB (‘severe generalized’, previously
called Hallopeau–Siemens: HS and ‘generalized other’). Soft tissue involvement. The oral mucosa of patients with generalized RDEB is reported to be extremely many friable and may slough off easily when touched45. Recurrent oral mucosal blistering is common, affecting almost all patients9,11,16,22,27,30,36,51,76. The blisters may be fluid- or blood-filled and arise at any oral mucosal surface, especially the tongue (Images 20–23).22 Denuded tongue. Tongue papillae are absent.4,5,7,9,18,22,28,30,36,41 (Image 24) Ankyloglossia. Ankyloglossia presumably secondary to ulceration is common, indeed may affect all patients (Image 25)1,4,5,7,12,16,18,19,22,23,28,31,77. Vestibule obliteration. The scarring of generalized RDEB can give rise to obliteration of the labial and buccal vestibules4,7,9,11,12,18,19,22,23,27,28,31,36,41 and hence has the potential to compromise oral hygiene procedures, dental treatment, and the wearing of removable prosthetic appliances (Image 26). Microstomia. Progressive5,78 microstomia affects almost all patients with generalized RDEB (Image 27)1,4-7,11,12,16,18,19,22,27,28,36,41,45,51,77. Microstomia is not unique to generalized RDEB, and it might also be present less severely in RDBE inversa and Herlitz subtype of JEB5,19.