Oral manifestations of epidermolysis bullosa dystrophica: a rare genetic disease

Epidermolysis bullosa (EB) constitutes a group of phenotypically diverse genodermatoses, which manifests with blistering and erosions of the skin and mucous membranes as the unifying diagnostic feature. It is an acquired disease or inherited as either autosomal-dominant or recessive with an incidenc...

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Veröffentlicht in:BMJ Case Reports 2013, Vol.2013
Hauptverfasser: Parushetti, Anita Dundappa, Agrawal, Jiwanasha Manish, Nanjannawar, Lalita Girish, Agrawal, Manish Suresh
Format: Report
Sprache:eng
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Zusammenfassung:Epidermolysis bullosa (EB) constitutes a group of phenotypically diverse genodermatoses, which manifests with blistering and erosions of the skin and mucous membranes as the unifying diagnostic feature. It is an acquired disease or inherited as either autosomal-dominant or recessive with an incidence of 1/50 000. The prominent clinical characteristic of the disease is the development of bullae or vesicles in mucosa or skin in response to minor trauma. It is a chronic mechanobullous disease characterised by auto antibodies against Type VII collagen. This paper documents a case of a man diagnosed with dominant dystrophic EB; describing the measures that dentists and healthcare providers should adopt in order to provide a safe and effective dental treatment. Early prevention protocols for these patients have also been discussed.
ISSN:1757-790X
DOI:10.1136/bcr-2012-007963