Hereditary Sensory Neuropathy: A Case Report

  • Ebru Saribas Dicle University, Faculty of Dentistry, Department of Periodontology, Diyarbakır, Turkey
  • Filiz Acun Kaya Dicle University, Faculty of Dentistry, Department of Periodontology, Diyarbakır, Turkey
  • Arzum Guler Dogru Dicle University, Faculty of Dentistry, Department of Periodontology, Diyarbakır, Turkey
  • Mehmet Ufuk Aluclu Dicle University, Faculty of Medicine, Department of Neurology, Diyarbakır, Turkey
Keywords: Neuropathy, hereditary sensory and autonomic neuropathy, HSAN, oral hygiene

Abstract

Aim: Hereditary sensory and autonomic neuropathy (HSAN) is a rare syndrome of unknown etiology that develops in early childhood. Five different types of HSAN have been described. This syndrome is characterized by the absence of pain and self-mutilation. Patients start to traumatize themselves at as young as 2–3 years of age. Subsequently, ulcers and stress fractures develop on their fingers and toes. Oral findings in HSAN patients include oral lesions after repetitive trauma, oral mucosa and tongue scars, self-dental extractions, dental infections, and premature tooth loss.

Methodology: This case report presents the oral findings of a 4-year-old HSAN patient with premature tooth loss who visited Dicle University, Faculty of Dentistry, Department of Periodontology. The extraoral examination showed wounds around the nose and bruises and burns on the forearms and legs. The intraoral inspection showed traumatic lesions on the tongue, early loss of all of the lower jaw primary dentition, but the presence of upper jaw teeth.

Results: During follow-up, the eruption of the permanent right lower incisors and presence of mobility were observed. A partial child prosthesis for the lower jaw and protective plaque restoration constructions were planned. The patient is being followed regularly.

Conclusions: It is necessary to conduct regular checkups and to ensure good communication between the dentist and family in terms of ensuring oral hygiene and preventing the development of undesired complications. 

How to cite this article: Saribas E, Acun Kaya F, Dogru AM, Aluclu MU.  Hereditary Sensory Neuropathy: A Case Report. Int Dent Res 2016;6:9-12.

Linguistic Revision: The English in this manuscript has been checked by at least two professional editors, both native speakers of English.

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Published
2016-04-15
How to Cite
Saribas, E., Acun Kaya, F., Dogru, A., & Aluclu, M. (2016). Hereditary Sensory Neuropathy: A Case Report. International Dental Research, 6(1), 9-12. https://doi.org/10.5577/intdentres.2016.vol6.no1.2