Segmental neurofibromatosis

Segmental neurofibromatosis or type V neurofibromatosis is a rare genodermatosis characterized by neurofibromas, café-au-lait spots and neurofibromas limited to a circumscribed body region. The disease may be associated with systemic involvement and malignancies. The disorder has not been reported y...

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Detalles Bibliográficos
Autores principales: Sobjanek, Michał, Dobosz-Kawałko, Magdalena, Michajłowski, Igor, Pęksa, Rafał, Nowicki, Roman
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Termedia Publishing House 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4293382/
https://www.ncbi.nlm.nih.gov/pubmed/25610358
http://dx.doi.org/10.5114/pdia.2014.40942
Descripción
Sumario:Segmental neurofibromatosis or type V neurofibromatosis is a rare genodermatosis characterized by neurofibromas, café-au-lait spots and neurofibromas limited to a circumscribed body region. The disease may be associated with systemic involvement and malignancies. The disorder has not been reported yet in the Polish medical literature. A 63-year-old Caucasian woman presented with a 20-year history of multiple, flesh colored, dome-shaped, soft to firm nodules situated in the right lumbar region. A histopathologic evaluation of three excised tumors revealed neurofibromas. No neurological and ophthalmologic symptoms of neurofibromatosis were diagnosed.

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