Indian Journal of Dermatology
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CASE REPORT
Year : 2010  |  Volume : 55  |  Issue : 1  |  Page : 105-108

Segmental neurofibromatosis: A report of 3 cases


1 Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha, India
2 Government Medical College, Nagpur, India

Correspondence Address:
Sushma Kashinath Gabhane
6-A, Forest Colony, Near New M.L.A, Hostel, Civil Lines, Nagpur - 440 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.60366

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Neurofibromatosis is a genetic disorder of neural crest-derived cells that primarily affect growth of neural tissues. It is broadly divided into three categories: (a) von Recklinghausen's neurofibromatosis or NF-1, (b) bilateral acoustic neuroma (NF-2), and (c) all other neurofibromatoses, including alternate or atypical forms of the disease. The patients with generalized form of NF1 are characterized by multiple café-au-lait spots and neurofibromas and diagnosed easily. But when an individual has small number of lesions in a limited region of the body it could be neglected by the patient or not be recognized by the clinicians as a segmental form of neurofibromatosis. We describe three cases of segmental neurofibromatosis (SNF). These cases have been classified as segmental NF according to Riccardi's definition of SNF and classification of neurofibromatosis. Segmental form of NF may evolve into a complete form over time. Also, this disorder may be transmitted to the offspring's of these individuals. Hence genetic counseling of these individuals must include these facts.


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