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Periodontal manifestations of von Recklinghausen neuro fibromatosis.

Shetty B, Umesh Y, Kranti K, Seshan H - J Indian Soc Periodontol (2013)

Bottom Line: 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 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Periodontics, M.S. Ramaiah Dental College and Hospital, Bangalore, Karnataka, India.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

Swelling of the right side of the mandible
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Figure 1: Swelling of the right side of the mandible

Mentions: A 40-year-old lady reported to the Department of Periodontics M.S.R.D.C from Department of General Surgery M.S.R.M.C with a history of multiple faint rounded densities in the skin, chest pain occasionally, since 8 months and breathlessness since 1 year. Patient's immediate family did no report of neurofibromatosis. Swelling of the right side of the angle of the mandible with limited mouth opening [Figure 1]. There was buccal and cortical bone enlargement extending from anteriorly up to canine and posteriorly up to retro molar region [Figure 2]. Well-defined swelling present in the right palatal region, which was firm in consistency measuring 2 cm × 1 cm approximately and ovoid surface and normal in color [Figure 3]. Areas of oral melanin pigmentation was also seen [Figure 4].


Periodontal manifestations of von Recklinghausen neuro fibromatosis.

Shetty B, Umesh Y, Kranti K, Seshan H - J Indian Soc Periodontol (2013)

Swelling of the right side of the mandible
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3713762&req=5

Figure 1: Swelling of the right side of the mandible
Mentions: A 40-year-old lady reported to the Department of Periodontics M.S.R.D.C from Department of General Surgery M.S.R.M.C with a history of multiple faint rounded densities in the skin, chest pain occasionally, since 8 months and breathlessness since 1 year. Patient's immediate family did no report of neurofibromatosis. Swelling of the right side of the angle of the mandible with limited mouth opening [Figure 1]. There was buccal and cortical bone enlargement extending from anteriorly up to canine and posteriorly up to retro molar region [Figure 2]. Well-defined swelling present in the right palatal region, which was firm in consistency measuring 2 cm × 1 cm approximately and ovoid surface and normal in color [Figure 3]. Areas of oral melanin pigmentation was also seen [Figure 4].

Bottom Line: 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 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Periodontics, M.S. Ramaiah Dental College and Hospital, Bangalore, Karnataka, India.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus