Clinical and Experimental Dermatology · 2004 · 44 citations · 20 references
DentistryOral PathologyPediatric DentistryChild Oral HealthMaxillofacial PathologyPediatric Dental CariesCaries EtiologyCongenital Oral AnomaliesDevelopmental Dental AnomaliesOral CareOral ConditionsPediatric Oral HygieneCaries Severity IndicesDental Caries SeverityEarly Childhood Caries ProgressionHereditary Dental DisordersOral Hygiene StatusOral InvolvementPediatric Oral LesionsPediatric Oral Pathology
Recessive dystrophic epidermolysis bullosa of Hallopeau-Siemens (RDEB-HS) is a rare genetic disorder characterized by trauma-induced blisters, milia, acral pseudosyndactyly, and scarring. RDEB-HS patients present with a distinct pattern of oral involvement consisting of microstomia, ankyloglossia, vestibule obliteration and dental caries. In this review, we describe the orodental manifestations of RDEB-HS and present our experience in a cohort of six new cases of RDEB-HS in children aged 6-10 years, documenting the presence of microstomia, ankyloglossia and vestibule obliteration in childhood. We also show that compared with unaffected control children, RDEB-HS subjects have a greater risk of developing high caries indices with early onset, both for permanent or deciduous teeth, and a worse oral hygiene index (scored as OHI). Tooth malpositions and the cross-bite relationship between maxilla and mandible could play a major role in promoting these events. We propose that dental management of RDEB-HS subjects should commence as soon as tooth eruption begins.
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