What is the disease of Recklinghausen

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  • Neurofibromas, she fibronevroma
  • Recklinghausen's disease



  • Neurofibromas, she fibronevroma

    What is the disease of Recklinghausen Typically, nerve tumor - thisbenign tumors affecting the main trunk of the nerve or its branches. Although any affected nerves, including spinal roots and cauda equina, most tumors are located subcutaneously as soft formations, sometimes red (in lesions of cutaneous nerves multiple elastic manifested easily palpable nodes).
    There are two main types of nerve tumors: schwannomas and neurofibromas.

    Neurofibromas (fibronevroma) - benignperipheral nerve tumor that develops from the cells and Schwann cells, called fibroblasts. Occasionally exposed to malignant transformation.

    Typically, nerve tumor - thisbenign tumors affecting the main trunk of the nerve or its branches. Although any affected nerves, including spinal roots and cauda equina, most tumors are located subcutaneously as soft formations, sometimes red (in lesions of cutaneous nerves multiple elastic manifested easily palpable nodes).

    Neurofibromas are more often multiple. They can ozlokachestvlyatsya. Neurofibroma - a characteristic feature of neurofibromatosis type I. In this disease the number of neurofibromas varies from one to several thousand.

    Neurofibromas often asymptomatic, but localization in a confined space (such as intervertebral hole) may cause neuropathy or radiculopathy.


    Neurofibromas may undergo malignant transformation in neyrofibrosarkomy.

    Recklinghausen's disease


    So, what is the disease of Recklinghausen?

    Neurofibromatosis type I, the disease orRecklinghausen is characterized by neurofibromas and focal hyperpigmentation of the skin - the so-called patches of color of coffee with milk. Identify at least six spots of color of coffee with milk more than 1.5 cm in diameter allows a diagnosis of neurofibromatosis type I.

    Other manifestations of the disease include:
    • benign tumors of the iris (called Lisch nodules)
    • like freckles small spots in the armpits,
    • pseudoarthrosis of the tibia.

    Furthermore, there may be:

    • hydrocephalus,
    • scoliosis,
    • short stature,
    • hypertension,
    • mental retardation.

    The cause of the disease is mutation of the gene, called NF1.


    In neurofibromatosis type I significantly increased the risk of developing diseases such as plexiform neurofibroma, optic nerve glioma, pheochromocytoma, meningioma, astrocytoma.

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