Oro-dental manifestations in Hallopeau-Siemens-type recessive dystrophic epidermolysis bullosa.
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.
De Benedittis
M
M
Department of Odontostomatology and Surgery, School of Dentistry, University of Bari, Bari, Italy. micdeben2002@libero.it
Petruzzi
M
M
Favia
G
G
Serpico
R
R
D002648:Child; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D009059:Mouth Diseases / Q000209:etiology*; D014076:Tooth Diseases / Q000209:etiology*