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Dermatology and Dermatologic Diseases
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Case Report

Association between Neurofibromatosis Type I and Central Giant Cell Lesion: Case Report

Monyque Cunha Trindade1, Fernando Melhem Elias2*, Rodrigo Chenu Migliolo1, Nathalia Cayuela Nogueira1 and Flávio Welington da Silva Ferraz3
1Department of Oral and Maxillofacial Surgery at the University Hospital of the University of Sao Paulo, Brazil
2School of Dentistry and Department of Oral and Maxillofacial Surgery at the University Hospital of the University of Sao Paulo, Brazil
3Department of Oral and Maxillofacial Surgery at the University Hospital of the University of Sao Paulo, Brazil
Corresponding Author : Fernando Melhem Elias
Associate Professor, School of Dentistry and University Hospital
Director, Residency Program on Oral and Maxillofacial Surgery
University of Sao Paulo – Brazil
Tel: +55-11-98294-2299
E-mail: [email protected], [email protected]
Received December 06, 2014; Accepted December 07, 2014; Published January 05, 2015
Citation:Trindade MC, Elias FM, Migliolo RC, Nogueira NC, Ferraz FWDS (2015) Association between Neurofibromatosis Type I and Central Giant Cell Lesion: Case Report. Pigmentary Disorders 2:161. doi:10.4172/2376-0427.1000161
Copyright: ©2015 Trindade MC, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract

Neurofibromatosis type I (NF1), or Von Recklinghausen disease, is characterized by café-au-lait spots and neurofibromas of the skin, bone defects, iris hamartomas (Lisch nodules) and tumors in the central nervous system. The central giant cell lesion (CGCL) is a non-neoplastic entity, intra-osseous, radiolucent, uni or multilocular, well-defined edges, and it can change the position of the teeth, but rarely causes resorption of them. The association between CGCL and NF1 has been described in the literature. Patient C.R.P, 42 years old, female, who has NF1 attended the service presenting asymptomatic swelling in the anterior maxilla and discrete painless intraoral bulge in the anterior mandible with approximate evolution of one year. Incisional biopsy was performed, and the result was CGCL. The patient didn’t have hyperparathyroidism. Treatment consisted of curettage of the lesion and bone regularization performed three years ago, without signs of recurrence. The occurrence of CGCL in multiple locations is uncommon and it is usually associated with hyperparathyroidism, cherubism, and can be also found in syndromes such as: Ramon Jaffe Campanacci, Noonan-like and NF1. The patient presented four features of NF1 (multiple skin neurofibromas, caféau- lait spots, Lisch nodules and freckles in the armpits) and CGCL in two locations, which corroborate the existence of this association in the literature. The apparent relationship between NF1 and an increased incidence of CGCL s in the jaws could represent a coincidental association, genetic relationship, or susceptibility to developing CGCL in abnormal bone quality. In cases of multiple CGCL, the presence of hyperparathyroidism, cherubism and syndromes such as NF1, should be considered. One realizes the importance of investigation of systemic factors and clinical signs for performing differential diagnosis. The differentiation of these lesions in aggressive and nonaggressive should be done to improve individual treatment plan. Surgical curettage is acceptable for the treatment of non-aggressive CGCL.

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