Oral Path: Hereditary Conditions Flashcards
White Sponge Nevus
Asymptomatic spongy white lesion on buccal mucosa
* Doesn’t wipe off
* Autosomal Dominant
Epidermolysis Bullosa
Skin & mucosa is fragile & Blisters easily
* Widespread blisteriing
Hereditary Hemorrhagic Telangiectasia (HHT)
AkA Olser-Weber-Rendu Syndrome
Abnormal Capillary Formation on skin, mucosa, and viscera
associated with:
* iron deficiency anemia
* Espistaxis (Nose bleeds)=frequent sign
Telangiectasia=red macule or papule from diluted or broken capillaries
Cleidocranial Dysplasia
Autosomal Dominant
Common Sign:
* Missing/poorly developed clavicles–shoulders appear hunched in towards midline
* Supernumery teeth
Tx: Alot of Extractoins & Dentures
Ectodermal Dysplasia
X-Linked Recessive
Common Signs:
* Missing Teeth
* Hypoplastic hair or nails
Amelogenesis Imperfecta
Intrinsic alteration of the ENAMEL in primary and permanent teeth
* Normal Dentin and Pulp
Autosomal Dominant, Recessive, or X-linked
Tx: Full coverage crowns for cosmetics
Dentinogenesis Imperfecta
Intrinsic alteration of DENTIN in primary & permanent teeth
Autosomal Dominant
Characteristics:
* SHOrt roots, bell shaped crowns, & obliterated pulps
* Bulbous crowns in radiographs (Bc constricted DEJ)
* Blue sclera
Tx: Full Coverage crowns
Regional Odontodysplasia
Quadrant of teeth exhibiting:
* short roots
* open apices
* enlarged pulp chambers
Radiographic:
* Ghost teeth: pulps are so huge they make the teeth look almost completely RL
Tx: Ext affected teeth
Dentin Dysplasia
Intrinsic alteration of dentin in permanent & primary teeth
Autosomal Dominant
2 types:
* Type 1: Chevron Pulp w/short roots
* Type 2: Thistle shaped Pulp w/normal roots; Primary teeth=blue or amber color
Tx: Not good candidates for restorations
Fusion & Gemination
Fusion:
* 2 tooth buds merging into 1 tooth
* Tooth count=1 less than normal
Gemination:
* 1 root buds into 2 crowns
* tooth count=normal
