Vascular Skin Lesions

Nevus Simplex

• 'stork bite'; pink macule, irreg border (distended/dilated capillaries)
• most common vascular birthmark; in up to 50% of nbn
• nape of neck, upper eyelids, nasal bridge, upper lip'
• blanching, prominent w/ crying
• fade by 2nd year; ones on nape may persist

Port Wine Nevus (Nevus Flammeus)

• flat pink/red/purple lesion w/ sharp edges (dilated, congested capillaries)
• jet black in AA infants
• non-blanching
• does not grow or get smaller, may be small or huge
• usually unilateral, most often on face
• RX: initially, cover w/ water repellent cosmetic cream, pulsed dye laser therapy
• if in pattern of trigeminal nerve (forehead upper eyelid), may be assoc w/ Sturge-Webber syndrome,which causes a prolif of endothelial cells, esp in small vessels: seizures, MR, hemiparesis, glaucoma. Unknown cause. Nonhereditary.

Strawberry Hemangioma

• bright red, raised, lobulated tumor
• soft, compressible, sharp margins
• head, neck, trunk, extremities
• in throat, may cause obstruction
• occurs in up to10% of nbns
• dilated capillaries and associated endothelial prolif in dermal/subdermal layers
• 20-30% present at birth, remainder apparent by 6 mos of age
• increases in size over 6 mos, regresses over several years
• complications: bleeding, ulceration, infxn, compression
• no tx, unless obstruction, consider systemic corticosteroids.

Cavernous Hemangioma

• blue-red skin color, soft compressible mass, poorly defined borders
• like strawberry hemangioma, but larger vessels, involving dermis and subq tissues
• increases in size over 1st year, then spontaneous regression
• tx if intefere w/ vital fn's - systemic corticosteroids
• 2 associated syndromes, mnemonic (K)avernous = KK

Kasabach-Meritt

• cavernous hemangioma associated with platelet sequestration and thrombocytopenia
• transfuse platelets prn. Corticosteroids.

Klippel-Trenaunay-Weber

• vascular nevus w/ hypertrophy of bone/soft structures of an extremity
• hypertrophy due to excess blood flow and malformed vessels in extremity
• rare. seen more often in males. px depends on severity of limb involvement.