Saratov JOURNAL of Medical and Scientific Research

phakomatosis

Neurofibromatosis in the practice of dermatology

Year: 2018, volume 14 Issue: №4 Pages: 760-763
Heading: Dermatovenerology Article type: Case report
Authors: Slesarenko N.A., Morrison A.V., Marchenko V.M., Moiseev A.A., Sherstobitova K.Yu., Ishmukhametova R.A.
Organization: Saratov State Medical University
Summary:

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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