What are the cutaneous manifestations of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

The hallmark of SWS is a facial cutaneous venous dilation, also referred to as a nevus flammeus or port-wine stain (PWS), which is present in as many as 96% of patients and is visible at birth (see the image below). The facial venous dilation appears as 1 or several dull red patches of irregular outline that are situated along, but are not limited to, the distribution of 1 or more divisions of the trigeminal nerve. [6, 7, 8] SWS belongs to a group of disorders collectively known as the phakomatoses ("mother-spot" diseases). (See Pathophysiology and Clinical Presentation.)

A child with Sturge-Weber syndrome with bilateral A child with Sturge-Weber syndrome with bilateral facial port-wine stain.

Laser therapy is available for the PWS. Although concerns have been raised that laser therapy to treat PWS might cause or worsen glaucoma or ocular hypertension; a 2009 retrospective review did not reveal evidence to support this. (See Treatment.) [2]


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