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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
CASE REPORT - LESIONS INVOLVING SOFT TISSUES Table of Contents   
Year : 2020  |  Volume : 24  |  Issue : 4  |  Page : 106-109
A rare case report of neurofibromatosis type 1 in a 12-year-old child: A 15-month follow-up


Department of Pedodontics and Preventive Dentistry, Panineeya Institute of Dental Sciences and Research Centre, Hyderabad, Telangana, India

Correspondence Address:
Srinivas Nallanchakrava
Department of Pedodontics and Preventive Dentistry, Panineeya Institute of Dental Sciences and Research Center, Hyderabad - 500 060, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jomfp.JOMFP_35_20

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Neurofibromatosis type 1 (NF-1) or von Recklinghausen's disease is a rare genetic disorder characterized by the development of multiple noncancerous (benign) tumors of nerves and skin (neurofibromas). Head-and-neck neurofibroma is generally located in the soft tissue. Here, we present a case of a 12-year-old girl with NF-1. The disease started in childhood with the appearance of multiple hyperpigmented skin macules. The girl presents generalized freckling and café au lait spots throughout the body and a diffused swelling measuring about 4 cm × 3 cm, extending from the right maxillary hard palate region to the midpalate. The diagnosis of NF-1 was made according to the presence of two or more diagnostic criteria of the National Institute of Health Consensus Development Conference. No recurrence was observed in a 15-month follow-up after extensive surgical ablation.


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Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
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