infantile hemangiomas most common vascular tumor of infancy 10% more common in caucasians females...
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Hemangiomas and Vascular Malformations
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Infantile HemangiomasMost common vascular tumor
of infancy10%
More common in CaucasiansFemalesPremature infantsPlacental abnormalities
Location >50% head and neck25% trunkRest on extremities
TimingSeveral days to weeks after
delivery
Hemangiomas
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Infantile HemangiomasDescription
Reddening or bluish discoloration of skin
Bright red nodule or plaque with elevation
TypesSuperficial (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
Hemangiomas
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Infantile HemangiomasCourse
Grow and peak by 6-9 months
Stabilization Involution
10% per yearGraying out of surface
40% with residual skin changesTelangiectasiasFibro-fatty tissue
Hemangiomas
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HemangiomatosisMultiple hemangiomasBenign
Limited to the skinNot benign
Numerous small (<2cm), widely dispersed cutaneous lesions
Internal or visceral lesionsLiver
May have AV shunts and precipitate high-output CHF 6-12 weeks of age
GI tract Bleeding
CNS Mass effect
Lungs
Hemangiomas
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When to worry?Lower face
Lower lip, chin, preauricular, neck
“beard” distributionAirway involvement
Midline lumbosacralSpinal dysraphism
Hemangiomas
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PHACES syndromePosterior fossa
malformationsHemangiomas
Plaque-like segmental hemangioma of the face
May initially be confused with port-wine stain
Often ulcerate and proliferate rapidly
Arterial anomaliesCarotid
Cardiac defectsEye anomaliesSternal clefting
Hemangiomas
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ComplicationsPeriorbital and lid lesions
Occlusion of the visual axis
Corneal compressionMust be treated
aggressivelyAmblyopia, strabismus,
astigmatismLips, nose or ears
High potential for disfigurement
High friction areasUlcerationSecondary infectionScarring
Hemangiomas
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TreatmentConservative management for mostLesions involving the airway or the eye
SteroidsInterferonSurgical intervention
Hemangiomas
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Common benign vascular tumorsOvergrowth 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
Pyogenic Granuloma
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Nevus Simplex AKA Salmon Patch or “Stork Bite”Capillary malformationSeen in majority of infants at birthLocation
Nape of neckGlabellaForeheadUpper eyelidsLower back
CourseFade with timeMore apparent when crying or
straining
Vascular Malformations
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Nevus FlammeusAKA Port-Wine stainCongenital capillary/venous
malformationDescription
Purple-redLocation
Unilaterally on faceCourse
Do not enlarge or involute
Vascular Malformations
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Sturge-Weber SyndromePort wine stain
Distribution of the trigeminal nerve
Vascular malformations of the ipsilateral leptomeninges and cerebral cortex
GlaucomaOther
Seizures, MR, hemiplegia
Klippel-Trenaunay SyndromePort wine stain
Over an extremityHemihypertrophy
Soft tissue and bony overgrowth
Vascular Malformations
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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 neviB. Congenital
nevomelanocytic neviC. Halo nevusD. Nevus sebaceousE. Ash-leaf spot
Question 9
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Nevi
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Congenital Nevomelanocytic NeviDescription
Pigmented plaques often associated with dense hair growth
CourseBirth
Tan or light pink with soft vellus hairs
Infancy and childhoodDarkening with small dark
macules or nodules within the plaque and prominent hair
Nevi
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Congenital Nevomelanocytic NeviSize
Small <1.5cmMedium 1.5-20cmLarge or Giant >20cm
PrognosisAll have potential for
malignant transformationNew, darker and/or bleeding
nodulesSudden growth1-4% small to medium10-30% Giant
Nevi
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Congenital Nevomelanocytic NeviManagement
Small to mediumYearly follow-up with dermExcision if atypical or difficult to monitor
GiantEarly, full thickness excision followed by grafting
ORClose observation every 6 months
Nevi
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Acquired Nevomelanocytic NeviTiming
Early childhoodDescription
Small, flat, pigmented macules1-2mm
LocationSun-exposed areas
CourseJunctional nevi
Limited to epidermal-dermal junctionCompound nevi
Papular or pedunculated Proliferation into the dermis
Change slowly over months and only warrant observation
Nevi
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Acquired Nevomelanocytic NeviHalo nevus
Hypopigmented or depigmented ring associated with mild local pruritus around a benign nevus
Caused by cytotoxic T-lymphocyte reaction
CourseEventual resolution and
nevus disappears
Nevi
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ChildhoodDe novoWithin giant congenital nevus or
other nevusTransplacental transfer
Red FlagsChange in size, shape or outline
Scalloped, irregular bordersChange in surface characteristics
Small, dark, elevated papule or nodule within a flat plaque
Flaking, scaling, ulceration or bleeding
Change in colorDifferent shade or to a mixture of
red, white or blueDevelopment of burning, itching
or tenderness
Melanomas
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Blue nevusSmall, firm, blue papuleDeep nevus cells
Traumatic hemorrhageUnder the nails or in mucous
membranesVascular lesions
Pyogenic granuloma or angiokeratoma
Spitz nevusRed and rapidly growing nevusComposed of spindle and
epithelial cellsConfused histologically with
melanoma
Melanoma Differential
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Epidermal NeviEpidermal structures onlyTiming
Birth or childhoodDescription
Slightly hyperpigmented papillomatous or verrucous growth
Increases in verrucous changes are common at puberty
Small and localized, linear, dermatomal or generalized
Other associations if extensiveSeizures, MR, ocular and
skeletal defects, hypophosphatemic vitamin D-resistant rickets
Hamartomatous Nevi
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Nevus sebaceous of Jadassohn Epidermal proliferation, abortive hair
follicles, sebaceous glands and apocrine structures
TimingPresent at birthMore 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
Hamartomatous Nevi