Infantile Hemangiomas Most common vascular tumor of infancy 10% More common in Caucasians Females Premature infants Placental abnormalities Location >50% head and neck 25% trunk Rest on extremities Timing Several days to weeks after delivery

Infantile Hemangiomas Description Reddening or bluish discoloration of skin Bright red nodule or plaque with elevation Types Superficial (epidermal) “Strawberry” or bright red Well demarcated Elevated Soft compressible Few mm to 5cm Deep (dermis or subq fat) Bluish hue Indistinct borders Doughy consistency Enlarge when dependent Mixed Most hemangiomas

Infantile Hemangiomas Course Grow and peak by 6-9 months Stabilization Involution 10% per year Graying out of surface 40% with residual skin changes Telangiectasias Fibro-fatty tissue

Hemangiomatosis Multiple hemangiomas Benign Limited to the skin Not benign Numerous small (<2cm), widely dispersed cutaneous lesions Internal or visceral lesions Liver May have AV shunts and precipitate high- output CHF  6-12 weeks of age GI tract Bleeding CNS Mass effect Lungs

When to worry? Lower face Lower lip, chin, preauricular, neck “beard” distribution Airway involvement Midline lumbosacral Spinal dysraphism

PHACES syndrome Posterior fossa malformations Hemangiomas Plaque-like segmental hemangioma of the face May initially be confused with port-wine stain Often ulcerate and proliferate rapidly Arterial anomalies Carotid Cardiac defects Eye anomalies Sternal clefting

Complications Periorbital and lid lesions Occlusion of the visual axis Corneal compression Must be treated aggressively Amblyopia, strabismus, astigmatism Lips, nose or ears High potential for disfigurement High friction areas Ulceration Secondary infection Scarring

Treatment Conservative management for most Lesions involving the airway or the eye Steroids Interferon Surgical intervention

Common benign vascular tumors Overgrowth of granulation tissue Following minor trauma Foreign body Timing Well after the newborn period Location Usually face or extremity Description Solitary bright red, soft nodules Pedunculated 5-6mm Friable surface Treatment Excision Electrodessication of the “feeder” vessels May recur

Nevus Simplex AKA Salmon Patch or “Stork Bite” Capillary malformation Seen in majority of infants at birth Location Nape of neck Glabella Forehead Upper eyelids Lower back Course Fade with time More apparent when crying or straining

Nevus Flammeus AKA Port-Wine stain Congenital capillary/venous malformation Description Purple-red Location Unilaterally on face Course Do not enlarge or involute

Sturge-Weber Syndrome Port wine stain Distribution of the trigeminal nerve Vascular malformations of the ipsilateral leptomeninges and cerebral cortex Glaucoma Other Seizures, MR, hemiplegia Klippel-Trenaunay Syndrome Port wine stain Over an extremity Hemihypertrophy Soft tissue and bony overgrowth

A new adolescent patient is seen in your office for a sports physical. He points out a hairless, well-circumscribed, yellowish waxy plaque located on his scalp. He says its been there since birth but has recently become more raised. He wants to know what it is? A. Epidermal nevi B. Congenital nevomelanocytic nevi C. Halo nevus D. Nevus sebaceous E. Ash-leaf spot

Congenital Nevomelanocytic Nevi Description Pigmented plaques often associated with dense hair growth Course Birth Tan or light pink with soft vellus hairs Infancy and childhood Darkening with small dark macules or nodules within the plaque and prominent hair

Congenital Nevomelanocytic Nevi Size Small <1.5cm Medium cm Large or Giant >20cm Prognosis All have potential for malignant transformation New, darker and/or bleeding nodules Sudden growth 1-4% small to medium 10-30% Giant

Congenital Nevomelanocytic Nevi Management Small to medium Yearly follow-up with derm Excision if atypical or difficult to monitor Giant Early, full thickness excision followed by grafting OR Close observation every 6 months

Acquired Nevomelanocytic Nevi Timing Early childhood Description Small, flat, pigmented macules 1-2mm Location Sun-exposed areas Course Junctional nevi Limited to epidermal-dermal junction Compound nevi Papular or pedunculated Proliferation into the dermis Change slowly over months and only warrant observation

Acquired Nevomelanocytic Nevi Halo nevus Hypopigmented or depigmented ring associated with mild local pruritus around a benign nevus Caused by cytotoxic T- lymphocyte reaction Course Eventual resolution and nevus disappears

Childhood De novo Within giant congenital nevus or other nevus Transplacental transfer Red Flags Change in size, shape or outline Scalloped, irregular borders Change in surface characteristics Small, dark, elevated papule or nodule within a flat plaque Flaking, scaling, ulceration or bleeding Change in color Different shade or to a mixture of red, white or blue Development of burning, itching or tenderness

Blue nevus Small, firm, blue papule Deep nevus cells Traumatic hemorrhage Under the nails or in mucous membranes Vascular lesions Pyogenic granuloma or angiokeratoma Spitz nevus Red and rapidly growing nevus Composed of spindle and epithelial cells Confused histologically with melanoma

Epidermal Nevi Epidermal structures only Timing Birth or childhood Description Slightly hyperpigmented papillomatous or verrucous growth Increases in verrucous changes are common at puberty Small and localized, linear, dermatomal or generalized Other associations if extensive Seizures, MR, ocular and skeletal defects, hypophosphatemic vitamin D-resistant rickets

Nevus sebaceous of Jadassohn Epidermal proliferation, abortive hair follicles, sebaceous glands and apocrine structures Timing Present at birth More verrucous, raised and nodular at puberty Description Hairless, well-circumscribed, skin-colored or yellowish waxy plaque Linear or round Location Scalp, face or neck Course Routine excision not recommended in childhood Malignancy risk is low May excise in adolescence or adulthood