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Sturge-Weber syndrome (SWS) is a sporadic, congenital disorder that impairs the development of the skin, eye and brain. Owing to its multisystem clinical manifestations, a multidisciplinary approach from dermatologists, neurologists and ophthalmologists is required. This article reviews data on pathogenesis, diagnosis and treatment of SWS with a focus on neurologic and dermatologic abnormalities. In terms of diagnosis, MRI, PET and electroencephalogram are discussed. Treatment of seizures includes prophylactic anti-epileptic medications, focal resection and hemispherectomy. Various treatment options for port-wine stains are also discussed, including pulsed-dye laser and new techniques, such as photodynamic therapy and the concurrent use of diagnostic imaging with pulsed-dye laser. Data supporting different theories of pathogenesis are also reviewed.
Dr. C. Lee, Vanderbilt University Medical Center, The Vanderbilt Clinic Division of Dermatology, 1301 22nd Avenue South, Nashville, TN 37232, USA. carla.lee@vanderbilt.edu