CHAPTER I
INTRODUCTION
Neurofibromatosis is an autosomal dominant disorder that affects the bone, nervous system, soft tissue, and skin. At least 8 different clinical phenotypes of neurofibromatosis have been identified that are associated with at least two genetic disorders. Clinical manifestations increase over time. Neurological and developmental problems toward malignancy might come afterwards.
Neurofibromatosis type 1 have varying phenotypic expression including dermatologic manifestations. Some patients may have a particular expression of the skin, while others may have life-threatening complications.
A neurocutaneous condition, neurofibromatosis may involve almost any organ system. Thus, the signs and symptoms can vary widely demonstrated. Two major subtypes exist: neurofibromatosis type 1, also known as von Recklinghausen neurofibromatosis, which is the most common subtype and is referred to as the neurofibromatosis, and neurofibromatosis type 2, the so-called central neurofibromatosis is a genetic disorder associated with bilateral vestibular schwannomas multisystem, schwannomas spinal cord, meningiomas, gliomas, and juvenile cataracts, skin with lack of features.
CHAPTER II
REVIEW REFERENCES
II. A. DEFINITION
Neurofibromatosis is a genetic disorder that interferes with the growth of cells in the nervous system, causing tumors to form in nerve tissue. These tumors can occur anywhere in the nervous system, including the brain, spinal cord, and nerves of large and small. Neurofibromatosis is usually diagnosed in childhood or early adulthood.
