Multiple talon cusps, dens in dente and concrescence in a child with hypomelanosis of Ito

Abstract

Hypomelanosis of Ito (HI) is a rare neurocutaneous disorder most likely caused by chromosomal mosaicism. HI patients may suffer from numerous clinical manifestations, but the expression of the disease is highly variable. Ophthalmologic, musculoskeletal, neurologic, and dental anomalies may be associated with the syndrome. The dental abnormalities found in HI include talon cusps, a single maxillary central in-cisor, enamel defects, hypodontia, and irregularly spaced teeth. The aim of this case report is to present multiple talon cusps, dens in dente and concrescence of maxillary permanent incisors in an 8-year-old boy affected with hypomelanosis of Ito. This unusual dental anomaly has been described in two previous reports of this neurocutaneous syndrome. HI can be difficult to diagnose, these dental findings may help to identify the syndrome in cases where other findings are minimal or atypical