Focal Epithelial Hyperplasia in a Child with Hereditary Sensory and Autonomic Neuropathy type IV: A Rare...

Hereditary sensory and autonomic neuropathies (HSAN) are a set of rare neurological conditions with five differing phenotypes. Type IV HSAN is notable for congenital insensitivity to pain. Focal epithelial hyperplasia (Heck's disease), a variant of human papillomavirus (HPV), presents as intraoral papules. The purpose of this report is to present the case of a five-year-old patient with HSAN type IV who was referred by her pediatric dentist to evaluate a tongue mass. Her medical history included recurrent urinary tract infections and verrucae on her hands and self-mutilation of the tongue and hands. Her dental history revealed that she had self-extracted 14 primary teeth. A soft tissue exam revealed flat surface papules on the tongue and a raised yellow, white and pink lesion on the right lateral tongue, which was diagnosed as focal epithelial hyperplasia. The co-occurrence of HSAN type IV and focal epithelial hyperplasia is a rare case that demonstrates the need for coordination among medical and dental professionals to achieve the optimal outcome. (J Dent Child 2019;86(3):154-7).