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International Journal of Advances In Case Reports

Volume 11, Issue 1, 2024
Mcmed International
International Journal of Advances In Case Reports
Issn
XXX-XXXX (Print), 2349 - 8005 (Online)
Frequency
bi-annual
Email
editorijacr@mcmed.us
Journal Home page
http://mcmed.us/about/ijacr
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Abstract
Title
VON-RECKLINGHAUSEN DISEASE: A CASE REPORT
Author
Dr. Arpan Aash, Dr. Arkaprava Banerjee, Dr. Mainak Datta, Dr. Madhurima Das
Email
aash.arpan1995@gmail.com
keyword
Neurofibromatosis, Lisch nodules, Café au lait Pigmentation, Crowe’s sign
Abstract
Von-Recklinghausen disease, also known as neurofibroma type 1(NF-1), is a benign peripheral nerve sheath tumor, which is one of the most frequent tumors of neural origin.. Neurofibromatosis type-1 occurs due to an alteration in the long arm of chromosome 17 and is an autosomal dominant inherited disease. There is no family history of the disease in about 50% of the NF-I patients. NF1 occurs in approximately one in 2,500-3,300 live births, regardless of the race, age, ethnic background but with slight female predilection. NF-I is characterized by the presence of skin lesions, bone malformations, and central nervous system tumors. A series of clinical criteria decide the diagnosis of NF-I. This article reports a case of in Von-Recklinghausen disease in a 45-year-old female patient with classical features of Crowe’s sign, Lisch nodules, café au lait spots along with intra-oral pyogenic granuloma and radiological changes of the mandible
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