Neurofibromatosis in the practice of dermatology

Автор: Slesarenko N.A., Morrison A.V., Marchenko V.M., Moissev A.A., Sherstobitova K.Yu., Ishmukhametova R.A.

Журнал: Саратовский научно-медицинский журнал @ssmj

Рубрика: Дерматовенерология

Статья в выпуске: 4 т.14, 2018 года.

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Neurofibromatosis belongs to the group of systemic hereditary diseases from the group of phakomatosis, characterized by developmental defects of ectodermal and mesodermal structures, mainly of the skin, nervous and bone systems, with an increased risk of developing malignant tumors. There are 7 types of the disease include a wide range of clinical manifestations even within the same family: a progressive course, the involvement of almost all organs and systems in the process, a high risk of complications, including many serious and fatal (cardiovascular) pulmonary insufficiency due to severe skeletal abnormalities, malignant degeneration by neurofibromas, etc.). An analysis of 5 clinical cases with neurofibromatosis is presented.

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Phakomatosis, neurofibromatosis (recklinghausen's disease), clinical case

Короткий адрес: https://sciup.org/149135187

IDR: 149135187

Статья научная