Congenital erythropoietic porphyria or Günther's disease along with a rare mandibular adenomatoid odontogenic tumor

Congenital erythropoietic porphyria (CEP), or “Günther disease,” is a rare variant of porphyria. It is an autosomal recessive disease caused by deficient uroporphyrinogen III synthase (URO-III-synthase), the fourth enzyme in the heme biosynthetic pathway. We report a case of a young female with the typical clinical presentations of cutaneous photosensitivity characterized by hyper- and hypo-pigmentations, blister formations and scarring of light-exposed skin, mutilation of the toe fingers, dark-purple urine and erythrodontia with pinkish fluorescence under a Wood's lamp. The diagnosis was confirmed by decreased activity of URO-III-synthase in red blood cells and a porphyrin profile compatible with CEP. Presence of adenomatoid odontogenic tumor associated with impacted mandibular right canine was also noted

Evaluation of influence of incisal guidance on the force exerted at the maximum possible intercuspal position and in protrusion – An In vivo study

Aim: Incisal guidance (IG) varies with maxillary and mandibular teeth wear. It in turn governs the forces on adhesive restorations. The present study evaluates the variation in forces because of changes in IG. Materials and Methods: IG was recorded on 46 subjects along with the distance from centric contact to incisal edge. Biteforce was recorded in the maximum possible intercuspal position (MIP) and in protrusion in the anterior region. Data analysis was performed using SPSS Version 21 at a confidence level ≤0.05. Pearson correlation test was applied to determine r value. Results: The association between forces on anterior teeth in MIP and forces on teeth in contact in protrusion is statistically significant (P value 0.001). There is definitive correlation between forces exerted in MIP and the distance travelled from centric contact to incisal edge (r = 0.059). There is a weak correlation between forces on anterior teeth in MIP and protrusive IG (r value 0.172). Conclusion: The force on anterior teeth in MIP with steeper IG is higher. Hence, the restoration margin should be kept away from the centric contact point