It is important to differentiate between the three most common
vascular anomalies in newborns: capillary stains that self-resolve,
port-wine stains that benefit from laser treatment, and hemangiomas,
which spontaneously involute.
The salmon patch is the most common benign vascular lesion seen
in infants, typically on the forehead, glabella, or nape of neck
that are most prominent during infancy, then self-resolve during
Synonyms Nevus simplex, telangiectatic
nevus, “stork bite,” nuchal nevus, Unna’s
nevus, evanescent macule, angel kiss, or aigrette.
Age Present at birth, fades with
Incidence Occurs in 30% to
40% of newborns.
Etiology Thought to be a persistence
of fetal circulation, gradually becomes less prominent.
Present at birth, these benign lesions fade with time. In lighter
skin types, the patch may be more persistent or evident during episodes
of crying or physical exertion. Fifty percent of salmon patches
in the nuchal region persist for life. They are asymptomatic and
Type Macular with telangiectasias.
Distribution Head and neck.
Sites of Predilection Nape of neck
(22%), glabella (20%) (Fig. 8-1A), and eyelids
Salmon patch A. Salmon
patch on the glabella of a newborn. B. Same
child at age 3 with no residual vascular lesion.
Salmon patch is the most common vascular birthmark. Its classic
locations and self-resolving tendencies should differentiate it
from other vascular birthmarks such as capillary malformations and hemangiomas.
Histopathology Skin biopsy reveals
dilated dermal capillaries.
Facial salmon patches fade with time (Fig. 8-1B) and only become
evident in lighter skin types with crying or physical exertion.
Nuchal salmon patches can persist but are asymptomatic and not usually
of cosmetic concern because they are covered by the posterior hairline.
Unlike capillary malformations, facial salmon patches fade almost
completely and usually do not require treatment. Persistent lesions
can occur in the nuchal area, but these are typically covered with
hair and not a cosmetic concern. Rare bothersome persistent lesions
can be treated with laser ablation.
(Port-Wine Stain) and Associated Syndromes
The most common capillary malformation is a port-wine stain (PWS):
a lesion comprising dilated capillaries, which are macular at onset,
but become plaquelike with time as the capillaries dilated. The
majority are present at birth, and unlike salmon patches, they persists
for life. PWS are benign, but rarely may be associated with a complicated
syndrome such as Sturge-Weber (see Table 8-1).
Table 8-1 Port-Wine Stains
and Associated Syndromes ||Download (.pdf)
Table 8-1 Port-Wine Stains
and Associated Syndromes
|Syndrome||Synonyms||Cutaneous Findings||Associated Features|
|Sturge-Weber syndrome||Encephalofacial angiomatosis||V1/V2 PWS, can have oral telangiectatic hypertrophy||Vascular malformation of ipsilateral meninges and cerebrum,
intracranial calcifications, seizures, hemiplegia, glaucoma, mental retardation|
|Klippel-Trenaunay syndrome||Nevus vasculosus hypertrophicus||PWS over a limb (leg much more commonly than arm), hemangiomas,
varicosities||Associated limb hypertrophy|
Synonyms Nevus flammeus, telangiectasia.
Age Present at birth, persists
Race Whites > Asians > blacks.
Etiology Mutation in vascular morphogenesis.
Given the occasional association of a PWS with Sturge-Weber or
Klippel-Trenaunay syndromes (see Table 8-1), it is postulated that
the PWS is caused by a mutation in the anterior neural crest or mesoderm
Type Infancy: macular. Adulthood:
Color Infancy: pink. Adulthood:
Shape Segmental. Large lesions
follow a dermatomal distribution and rarely cross the midline (Fig.
Port-wine stain A. Capillary
malformation on the trunk of an infant. B. Facial
capillary malformation in an older child.
Distribution Localized or diffuse.
Most commonly involve the face (Fig. 8-2B) in a trigeminal nerve
distribution (V1, V2, or V3).
The diagnosis of a PWS is made clinically. The differential diagnosis
of a PWS includes a hemangioma, salmon patch, venous malformation,
lymphatic malformation, or arteriovenous malformation. Doppler ultrasound,
CT, MRI, or other imaging studies may be needed if the diagnosis
Histopathology Dilated capillaries
and increased ectasias in the deep reticular dermis.
differentiating PWS from hemangiomas.
PWS do not regress spontaneously. The area of involvement tends
to increase in proportion to the size of the child. In adulthood,
PWS can turn darker red–purple in color and thicken, which
leads to more severe cosmetic disfigurement. In some cases, the
thickening is associated with hyperplastic skin changes and asymmetric
overgrowth of the area underlying the PWS (face > trunk and limbs).
Isolated PWS are benign but can be disfiguring and distressing
to the patient. Multiple treatments with a pulsed dye laser are
very effective and should be considered in childhood before the
lesion progresses to a more severe nodular, disfiguring
form. Alternatively, a PWS can be covered up with makeup.
The majority of PWS have no associated abnormalities. Infrequently,
a midline PWS of the lumbosacral, back, or nape area may be a hallmark
for spinal dysraphism especially if seen in conjunction with other
skin signs (pit, dimple, sinus, fibroma, lipoma, or hypertrichosis).
Dysraphism can be detected with ultrasound or MRI.
Rarely, PWS can be associated with Sturge-Weber syndrome or Klippel-Trenaunay
syndrome (see Table 8-1). It is estimated that 10% to 15% of
infants with a V1 PWS will develop the ocular glaucoma and neurologic
seizures of Sturge-Weber syndrome. If suspected, internal imaging
with radiography (calcifications), CT (pial brain lesions), MRI
(myelination), SPECT (blood flow), or PET (glucose metabolism) may
be helpful. Klippel-Trenaunay syndrome is the association of limb
hypertrophy with an overlying PWS or capillary venous malformation.
Again, Doppler ultrasound or MRI can better detect the extent of
tissue involvement in these patients.
and Associated Syndromes
Hemangiomas are benign, vascular proliferations that rapidly
enlarge during the first year of life and slowly spontaneously involute
by age 5 to 10 years. Superficial hemangiomas have a bright red,
nodular surface and deeper lesions may be blue–purple in
color. Rarely, they may be associated with systemic malformations
(see Table 8-2).
Table 8-2 Hemangiomas
and Associated Syndromes ||Download (.pdf)
Table 8-2 Hemangiomas
and Associated Syndromes
|Disorder||Synonyms||Cutaneous Findings||Associated Features|
|Rapidly involuting hemangioma||RICH||Hemangioma proliferates in utero, involutes first year of
|Noninvoluting congenital hemangioma||NICH||Hemangioma proliferates in utero, never involutes|
|Diffuse neonatal hemangiomatosis||Multiple hemangiomas||Hemangiomas of GI tract, liver, CNS, lungs|
|Benign neonatal hemangiomatosis||Multiple hemangiomas|
|PHACES syndrome||Large cervicofacial hemangioma||Posterior fossa abnormalities (Hemangiomas) Arterial abnormalities,
Cardiac defects, Eye anomalies, Sternal defects|
Synonyms Infantile hemangioma,
hemangioma of infancy, strawberry nevus, angioma cavernosum, capillary
Age 10% to 12% have
onset <1 years. Rarely present at birth.
Prevalence Seen more in premature
infants (<30 weeks gestational age or birthweight <1500 g),
infants of mothers status postchorionic villus sampling, infants
of older mothers, and infants of multiple gestation.
Race More common in Caucasians.
Incidence Most common tumor of
infancy. Seen in 2.6% of all newborns.
Etiology Abnormally increased vascular
proliferation. Reports of familial cases (?AD inheritance).
Hemangiomas are localized proliferations of blood vessels. Extensive
study is underway to understand the signaling mechanisms that cause
this benign tumor to grow, plateau, and then spontaneously involute.
Several proposed mechanisms for hemangioma formation include the
1. A mutation in endothelial cells,
2. A mutation in other cells influencing endothelial proliferation,
3. Placental origin of proliferative cells, and/or
4. Dysregulation of immature endothelial progenitor cells.
It seems that a combination of these mechanisms, multiple genes,
and local effects all influence the development, growth, and involution
Lesions present soon after birth during the first few weeks of
life. The lesions proliferate 6 to 18 months, and then spontaneously
involute by age 9 years; 30% of lesions involute by age
3, 50% by age 5, 70% by age 7, and 90% by
age 9. At the end of involution, most lesions are cosmetically undetectable,
but a few can leave atrophic, fibrofatty, or telangiectatic markings.
Rarely, a hemangioma can proliferate in utero, is present at birth
and undergoes rapid spontaneous resolution (“rapidly involuting
congenital hemangioma” or “RICH”) in
the first year of life. Even more rare is a noninvoluting congenital
hemangioma (“NICH”), which will not go away spontaneously. Both
of these latter hemangioma variants are the exception rather than
Solitary hemangiomas are asymptomatic and benign and rarely bleed
(see Fig. 8-3A). Possible complications include the following:
Hemangioma, infancy A. An
infant with a vascular lesion on the lower lip noted at birth. Ulcerated hemangioma in an infant B. An
infant with multiple hemangiomas and one ulcerating behind the ear. Resolved hemangioma in a child C. Involution
of a hemangioma has left a faint reticular vascular pattern.
1. Ulceration (seen in 10% of hemangiomas,
see Fig. 8-3B) most common in lip, neck, or anogenital locations.
2. Location near critical structures (periorbital, nasal tip,
lip, pinna, breast, and anogenital area) causing obstruction.
3. Associated with internal abnormalities (CNS, laryngeal obstruction,
and spinal dysraphism).
Type Nodule, plaque, may ulcerated.
Color Superficial: pink, red; Deep:
blue–purple. Involuting: white/gray.
Frequency 50% to 60% superficial,
25% to 35% combined, 15% deep.
Size Average 2 to 5 cm but can
grow up to 20 cm in size.
Pattern Focal or segmental.
Palpation Superficial lesions are
soft/compressible; deeper lesions are more firm.
Sites of Predilection Face, trunk,
legs, oral, and genital mucous membranes.
Hemangiomas are sometimes confused with other vascular abnormalities
such as capillary malformations, infantile myofibromatosis, or pyogenic
granulomas. Deeper lesions can mimic dermal or subcutaneous masses
such as fibrosarcoma, rhabdomyosarcoma, neuroblastoma, dermatofibrosarcoma
protuberans, nasal glioma, lipoblastoma, venous, lymphatic, or AV
malformations. The characteristic proliferative and involuting phase
can clinically differentiate hemangiomas from other vascular lesions.
Radiographic studies or histologic examination may be necessary
in most difficult cases.
Histopathology Proliferating: endothelial
cell hyperplasia, lobule formation, masts cells, and prominent basement membrane.
Involuting: fibrofatty tissue, decreased mast cells.
staining differentiating hemangiomas from all other vascular malformations.
Doppler Evaluation Can determine
slow flow hemangiomas versus fast flow AV malformations.
CT Scan Uniformly enhancing soft
tissue mass with dilated feeding and draining vessels.
MRI Soft tissue mass which is iso-or
hypointense to muscle with flow voids.
Hemangiomas spontaneously involute by age 5 to 9 years. The residual
skin changes are barely detectable but can include: skin atrophy,
fibrofatty tissue, telangiectasia, or depigmentation (Fig. 8-3C).
Typical spontaneous involution leaves the best cosmetic results,
and thus nonintervention in uncomplicated lesions is recommended.
Focal hemangiomas are typically small, arise from one locus and
spontaneously involute without any complications. Larger segmental
hemangiomas may be associated with systemic anomalies such as spinal
dysraphism, GI, GU anomalies, or PHACES syndrome (see Table 8-2).
A midline hemangioma of the lumbosacral, back, or nape area may
be a hallmark for spinal dysraphism especially if seen in conjunction
with other skin signs (pit, dimple, sinus, fibroma, lipoma, or hypertrichosis)
or other malformations (imperforate anus, GI fistulae, skeletal,
and renal abnormalities). An MRI can detect these underlying defects.
Ten to twenty-five percent of patients will have multiple hemangiomas,
and numerous hemangiomas can be a marker for internal involvement.
A child with numerous cutaneous hemangiomas should be evaluated
for the possibility of hemangiomas of the visceral organs, GI tract,
liver, CNS, lungs, mucous membranes, or eyes. Complications include
cardiac failure, GI bleeding, hydrocephalus, visceral hemorrhage,
and ocular abnormalities. Radiologic evaluation (ultrasound, CT,
or MRI) may be helpful in determining the extent of internal involvement.
Treatment for the majority of simple hemangiomas is unnecessary
because they are asymptomatic and self-resolve with a good cosmetic
Less than 2% of hemangiomas require intervention (for
ulceration/bleeding, blocking facial structures or GI/GU
tracts). Ulcerations can be managed with local wound care: saline
soaks, topical antibiotics (mupirocin or metronidazole), and occlusive
dressings. Pain with the ulcers can be alleviated with oral acetaminophen.
Superinfected lesions may require a course of oral antibiotics (such
as a first-generation cephalosporin).
Intralesional steroids can be used to treat small hemangiomas,
but periocular injections can be complicated by ophthalmic artery
occlusion and can lead to blindness. Topical steroids are safer, but
their efficacy is unclear. Topical 5% imiquimod cream reportedly
helps proliferating hemangiomas, but the cream can cause erosions
as a complication.
Systemic steroids, such as prednisolone or prednisone, are the
first-line treatment for life-threatening obstructive hemangiomas;
2–3 mg/kg/d is recommended until cessation
of hemangioma growth or shrinking is achieved, then the steroids
should be tapered. Adverse reactions to systemic steroid include
cushingoid facies, irritability, GI symptoms, and decreased growth
Systemic α-IFN (angiogenesis inhibitor) can be used for refractory,
severe life-threatening hemangiomas at a dose of 1–3 million
U/m/d injected subcutaneously. Adverse
effects include spastic diplegia, fever, irritability, diarrhea, neutropenia,
elevated LFTs, and skin necrosis. Systemic vincristine is a chemotherapy
approach for resistant life-threatening hemangiomas, but its effectiveness
is unclear. Adverse effects include peripheral neuropathy, jaw pain,
anemia, and leukopenia.
Pulsed dye laser is effective in lightening the surface of proliferative
red lesions; however, there was no change in clearance compared
to un-lasered children by age 1 and the PDL-treated children were
more likely to develop atrophy, hypopigmentation, ulceration, and
scarring. Argon and Nd:YAG lasers are capable of treating deeper
hemangiomas, but the risk of scarring is even higher with these
Surgical management (with or without embolization) is difficult,
and should be reserved for proliferating hemangiomas in life-threatening
locations. Otherwise the timing of surgical intervention might be
most helpful after complete involution (for any residual fibrofatty
component), for cosmetically it may leave a preferable cosmetic
outcome to surgical scarring.