Open AccessPeriodontal manifestations of von Recklinghausen neuro fibromatosis
Author(s) -
Bhavya Shetty,
Y Umesh,
K Kranti,
Hema Seshan
Publication year - 2013
Publication title -
journal of indian society of periodontology (print)/journal of indian society of periodontology (online)
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.309
H-Index - 28
eISSN - 0975-1580
pISSN - 0972-124X
DOI - 10.4103/0972-124x.113092
Subject(s) - medicine , neurofibromatosis , neurofibroma , dermatology , neurofibromatosis type i , lesion , expressivity , family history , disease , pathology , surgery , genetics , biology
Neurofibroma is an uncommon benign tumor of the oral cavity derived from the cells that constitute the nerve sheath neurofibromatosis type 1 (NF1), also known as von Recklinghausen's disease, is the most common type of neurofibromatosis and accounts for about 90% of all cases. It is one of the most frequent human genetic diseases, with the prevalence of one case in 3,000 births. Neurofibroma is seen either as a solitary lesion or as part of the generalized syndrome of neurofibromatosis. The solitary form does not differ from the disseminated form or the multiple form of the disease, except that systemic and hereditary factors present in the disseminated form are absent in the solitary type. Oral cavity involvement by a solitary and peripheral plexiform neurofibroma in patients with no other signs of neurofibromatosis is uncommon. The expressivity of NF1 is extremely variable, with manifestations ranging from mild lesions to several complications and functional impairment. Oral manifestations can be found in almost 72% of NF1 patients. This is a case report of a 40-year-old lady with a history of multiple faint rounded densities in the skin, chest pain occasionally since 8 months and breathlessness since 1 year and swelling of the right side of the angle of the mandible with limited mouth opening.