Conditions / Syndrome

neurofibromatosis 1

info · Syndrome · ICD-10: Q85.01

A neurofibromatosis characterized by multiple cafe-au-lait macules, skin fold freckling, neurofibromas, optic gliomas, Lisch nodules or choroidal abnormalities in the eye, or a specific bone abnormality that has_material_basis_in the NF1 gene on chromosome 17q

A neurofibromatosis characterized by multiple cafe-au-lait macules, skin fold freckling, neurofibromas, optic gliomas, Lisch nodules or choroidal abnormalities in the eye, or a specific bone abnormality that has_material_basis_in the NF1 gene on chromosome 17q11.2. Bone abnormalities include a distinctive osseous lesion such as sphenoid dysplasia, anterolateral bowing of the tibia, or pseudarthrosis of a long bone.

Signs and symptoms

  • Glioma
  • Pheochromocytoma
  • Inguinal freckling
  • Multiple cafe-au-lait spots
  • Few cafe-au-lait spots
  • Axillary freckling
  • Freckling
  • Neurofibroma
  • Specific learning disability
  • Mild intellectual disability

Medications that may treat it

selumetinib

Also known as: NF1; Peripheral Neurofibromatosis; Recklinghausen's neurofibromatosis; neurofibromatosis type I; von Recklinghausen Disease