2025 – PAGE 153 – DERMATOLOGY
PORT WINE STAINS (PWS) (AKA NEVUS FLAMMEUS)
Port Wine Stains (PWS), AKA nevus flammeus, are CAPILLARY malformations. They tend to be unilateral and segmental, not crossing the midline. They start as pink/flat lesions that become dark red-purple. They then progress to being thick/raised in adulthood. These PWSs are Present at birth and are PERMANENT. They are benign if noted in isolation. If noted on the face, they can be associated with glaucoma (increased intraocular pressure that can present as a red eye).
IMAGE: www.pbrlinks.com/PORTWINE1
IMAGE: www.pbrlinks.com/PORTWINE2
PEARL: They grow in proportion to the child and tend to occur in a segmental distribution respecting the midline.
MNEMONIC: Glaucoma is a concern if a PWS is noted in the facial area. Is that why Mikhail Gorbachev wore glasses? Because he has that big FLAME on his head?
STURGE-WEBER SYNDROME (SWS)
The Sturge-Weber Syndrome (SWS) includes the following findings: Port Wine Stain (PWS or NEVUS FLAMMEUS) + EYE/TRIGEMINAL NERVE DISTRIBUTION + INTRACRANIAL VASCULAR MALFORMATION (look for with MRI) +/- glaucoma +/- Seizures +/- cognitive deficits.
MNEMONICS: “pWS = sWS”… Ever heard of a basketball player named Chris WEBBER? Think WEBBER = Sports = ESPN (I know it’s a stretch).
- EYE – glaucoma
- SWS
- PWS
- NEUROLOGIC issues: Developmental delay, Seizures
CAPILLARY MALFORMATION ASSOCIATIONS
(DOUBLE TAKE) KLIPPEL-TRENAUNAY SYNDROME
Klippel-Trenaunay syndrome is associated with AV fistulae, causing skeletal or limb OVERGROWTH (hemihypertrophy). Patients with Klippel-Trenaunay have Port Wine Stains and overgrowth of tissue, bones, and soft tissue. Look for unilateral limb overgrowth and CHF.
- IMAGE: www.pbrlinks.com/KLIPPELTRENAUNAY1
- (DOUBLE TAKE) PEARL: Hemihypertrophy images on the pediatric exam should very quickly clue you in to a few disorders. Highest on your differential should be Beckwith-Wiedemann Syndrome, then Klippel-Trenaunay, then Russell-Silver Syndrome, and then possibly Proteus Syndrome.
- MNEMONIC: From now on, say CRIPPLE-T. Think of these patients as having a CRIPPLING disorder in which they have one HUGE leg that prevents them from getting around.
- NAME ALERT: KLIPPEL-FEIL SYNDROME. This is a completely different disorder. Look for a Torticollis-like photograph (due to fused cervical vertebrae).