Neurocutaneous Syndrome

Aka: Neurocutaneous Syndrome, Neurocutaneous

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II. Definitions

  1. Neurocutaneous Syndrome
    1. CNS syndromes associated with dermatologic findings

III. Pathophysiology

  1. Familial disorders of ectoderm differentiation

IV. Causes: Neurocutaneous Conditions

  1. Neurofibromatosis
    1. Cafe-Au-Lait Spot (Hyperpigmented Macule)
    2. Neurofibroma (soft Papules and Nodules)
  2. Tuberous Sclerosis
    1. Ash Leaf spots
  3. Sturge-Weber Disease
  4. Von Hippel-Lindau Disease
  5. Ataxia Telangiectasia
  6. Linear Nevus Syndrome
  7. Hypomelanosis of Ito
  8. Incontinentia pigmenti

V. References

  1. Behrman (2000) Nelson Textbook of Pediatrics, p. 1835
  2. Kandt (2002) Neurol Clin 20(4):941-64 [PubMed]

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