Localized aggressive periodontitis and childhood hypophosphatasia with concomitant external root resorption: is it a cause and effect relationship?

Introduction: Localized Aggressive Periodontitis (LAgP) is characterized by the early age of onset, the rapid rate of alveolar bone destruction and localized molar-incisor involvement. Till date, there are very rare evidences of LAgP occurring along with external root resorption (ERR) with underlying systemic disorder. Presented case report highlights the severe ERR that occurred along with LAgP in the presence of an underlying systemic disorder -Childhood Hypophosphatasia.
Childhood hypophosphatasia is detected at a later age with a consistent feature of premature loss of primary teeth without evidence of a significant inflammatory response. Teeth may show enlarged pulp chamber and significant degree of alveolar bone loss may be seen.
Case Report: An 18 year old male patient presented with the complaint of poor aesthetics and replacing his missing anterior teeth. There was no clinically detectable physical abnormality. Clinical examination of the oral cavity was conducted along with biochemical, haematological and radiological investigations. Radiographs revealed vertical bone loss (arc shaped) and severe external root resorption with teeth 26, 36 and 46. Biochemical investigations presented with a just below normal serum profile for alkaline phosphatase, parathormone and serum calcium suggestive of Hypophosphatasia (Childhood Form). No similar family history was reported. A diagnosis of localized aggressive periodontitis was established. However, there were no local or systemic disorders detected that could cause such severe amount of external root resorption. Histopathology report of Extracted molar showed rough cemental surface with hypo-plastic dentinal tubules suggestive of Hypophosphatasia.The differential diagnoses for the etiology of root resorption were LAgP & hypophosphatasia (Childhood form).