Acquired neurocutaneous disorders

Handb Clin Neurol. 2015;132:29-73. doi: 10.1016/B978-0-444-62702-5.00003-2.


A variety of neurologic diseases have cutaneous manifestations. These may precede, coincide with, or follow the neurologic findings. An array of autoimmune, genetic, and environmental factors play a role in expression and severity of the neurologic burden in these conditions. This chapter emphasizes congenital and genetic disorders, but we also discuss the pathophysiology and manifestation of various acquired neurocutaneous disorders with an emphasis Behcet's disease, dermatomyositis, Sjögren's syndrome, systemic lupus erythematosus, scleroderma, Parry-Romberg syndrome and Degos disease.

Keywords: Behçet's disease; Neurologic manifestations; Parry–Romberg syndrome and Degos disease; Sjögren syndrome; dermatologic manifestations; dermatomyositis; scleroderma; systemic lupus erythematosus (SLE).

Publication types

  • Review

MeSH terms

  • Behcet Syndrome / physiopathology
  • Congenital Abnormalities / physiopathology*
  • Dermatomyositis / physiopathology
  • Genetic Diseases, Inborn / complications*
  • Humans
  • Neurocutaneous Syndromes / complications*
  • Neurocutaneous Syndromes / genetics*
  • Sjogren's Syndrome / physiopathology