Oral Manifestation of Von Recklinghausen’s Disease: A Case Reports of Two Cases
Bismita Karki
Department of Dentistry, Kantipur Dental College, Nepal.
Sania Naz
Faculty of Dental Sciences, IMS, BHU, Varanasi, India.
Aman Kumar Mishra
Faculty of Dental Sciences, IMS, BHU, Varanasi, India.
Shakti Kumar Nayak
Department of Oral Pathology, Kothiwal Dental College & Research Centre, Moradabad, India.
Sabina Chhuju
Department of Dentistry, Nobel Medical College Teaching Hospital, Biratnagar, Nepal.
Peeyush Shivhare *
Department of Dentistry, Baba Kinaram Autonomous State Medical College, Chandauli. (U.P.), India.
*Author to whom correspondence should be addressed.
Abstract
Background: Von Recklinghausen’s disease also termed as “Neurofibromatosis type 1 (NF1)” is an autosomal dominant inherited disorder, characterized by various abnormalities such as neurofibroma, mainly in the skin and nerves. The classic manifestations include neurofibroma, café-au-lait macules, Iris Lisch nodules, optic glioma, axillary or inguinal freckling, and osseous dysplasia, neurological or cognitive impairment, scoliosis and oral and maxillofacial abnormalities. Various oral manifestations include soft tissues neurofibroma, dental abnormalities and osseous abnormalities.
Case Presentation: We are reporting two cases of von Recklinghausen’s disease with predominant osseous abnormalities in one case while soft tissue neurofibroma on another case.
Conclusion: Oral manifestations are not uncommon with NF1, including soft tissue, dental and osseous abnormalities. Oral specialist must be aware regarding the osseous and dental manifestation so that special attention and care must be provided to avoid further loss of dentition.
Keywords: Recklinghausen’s disease, autosomal, neurofibroma, optic glioma