Newborn Skin:​Part II.

Birthmarks
Katherine A. M. Snyder, MD, Mosaic Community Health–Madras Health Center, Madras, Oregon
Adam D. Voelckers, MD, University of Pittsburgh Medical Center, Lititz Family Medicine Residency Program, Lititz, Pennsylvania

Birthmarks in newborns can be classified as vascular, melanocytic or pigmented, or markers of underlying developmental
abnormalities of the nervous system. A nevus simplex is a benign capillary malformation. Newborns with a nevus flammeus
can be safely treated before one year of age with a pulsed dye laser to reduce the visibility of lesions. Infantile hemangiomas
should be treated with systemic beta blockers if there is a risk of life-threatening complications, functional impairment,
ulceration, underlying abnormalities, permanent scarring, or alteration of anatomic landmarks. Dermal melanocytosis is a
benign finding that is easily recognized and does not warrant further evaluation. A solitary congenital melanocytic nevus
that is less than 20 cm in diameter may be observed in primary care;​children with larger or multiple nevi should be referred
to pediatric dermatology due to the risk of melanoma. Newborns with skin markers of occult spinal dysraphism (other than a
simple, solitary dimple) should have lumbar spine imaging using ultrasonography or magnetic resonance imaging. (Am Fam
Physician. 2024;​109(3):217-221. Copyright © 2024 American Academy of Family Physicians.)

Birthmarks are common sources of parental concern, NEVUS SIMPLEX

and some require evaluation and treatment. Birthmarks A nevus simplex is colloquially known as a stork bite, angel
can be classified as vascular, as melanocytic or pigmented, kiss, or salmon patch, and is present in 82% of newborns.
or as markers of underlying developmental abnormalities These lesions present as pale pink to bright red or violet mac-
of the nervous system. Part II of this article reviews the ules with indistinct borders that can be partially blanched
identification and management of birthmarks that appear with pressure on the skin (Figure 1). Most are benign and
in the neonatal period, with an emphasis on prognosis and spontaneously regress by one year of age. Rarely, a nevus
appropriate counseling for parents. Part I of this article, simplex can be associated with genetic syndromes includ-
which also appears in this issue of American Family Physi- ing Beckwith-Wiedemann syndrome, port-wine nevi-mega
cian, discusses the presentation, prognosis, and treatment cisterna magna-hydrocephalus syndrome (Nova syndrome),
of common rashes and skin changes that may present odontodysplasia, macrocephaly-capillary malformation
during an infant’s first four weeks of life.1 syndrome, and Roberts-SC phocomelia syndrome.2 Neu-
rologic symptoms, seizures, large head size, and limb mal-
Vascular Lesions formations should prompt consideration of referral for
Vascular lesions result from abnormally formed blood genetic evaluation.
vessels. Capillary malformations, such as a nevus simplex
and nevus flammeus, are the most common presentations. NEVUS FLAMMEUS
Cutaneous vascular lesions can be associated with genetic A nevus flammeus, colloquially known as a port-wine
syndromes or extracutaneous malformations. Early recog- stain, occurs in 3 to 5 per 1,000 live births and is most
nition, diagnosis, and treatment of syndromes associated commonly present on the limbs or face. These lesions are
with vascular lesions in infants can significantly impact well-demarcated, pink or red lesions composed of abnor-
quality of life. mally dilated capillaries and venules.3 Lesions on the limbs
or trunk remain stable or lighten over time, whereas facial
lesions tend to become darker, taking on the port-wine
This is part II of a two-part article on newborn skin. Part I,
color.2 A nevus flammeus that involves the forehead may
“Newborn Skin: Common Rashes and Skin Changes,”
appears in this issue of AFP on page 212. indicate Sturge-Weber syndrome, a congenital neurocuta-
CME This clinical content conforms to AAFP criteria for CME. neous syndrome characterized by vascular malformations
See CME Quiz on page 205. in the brain or eyes;​therefore, ophthalmologic and neuro-
Author disclosure:​No relevant financial relationships. logic evaluations are recommended.4 Nevi flammeus can
progress to include nodules, pyogenic granulomas, and

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 NEWBORN SKIN: BIRTHMARKS
SORT:​KEY RECOMMENDATIONS FOR PRACTICE

Evidence
Clinical recommendation rating Comments
Newborns with a nevus flammeus that involves the forehead should be C Expert consensus statement
referred for ophthalmologic and neurologic evaluations.4

For infantile hemangiomas that have high-risk features, propranolol at a A Cochrane review
dosage of 2 to 3 mg per kg per day is the most effective treatment.10,15

Patients with a solitary congenital melanocytic nevus with a projected size C Expert opinion
of greater than 20 cm in any dimension or multiple congenital melanocytic
nevi of any size should be referred to dermatology.18

Newborns with lumbosacral findings such as hypertrichosis, hemangiomas, B Prospective and retrospec-
or a subcutaneous mass (e.g., lipoma) or caudal appendage, and those with tive clinical case reviews and a
multiple lumbosacral skin abnormalities should be evaluated for occult spi- cost-effectiveness analysis model
nal dysraphism with ultrasonography or magnetic resonance imaging. 21,23-25

Newborns with a solitary, atypical lumbosacral dimple should be evaluated B Prospective and retrospec-
for occult spinal dysraphism using ultrasonography. 21,23-25 tive clinical case reviews and a
cost-effectiveness analysis model

A = consistent, good-quality patient-oriented evidence;​B = inconsistent or limited-quality patient-oriented evidence;​C = consensus,

disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://​
www.aafp.org/afpsort.

FIGURE 1 FIGURE 2

Infantile hemangioma presenting as telangiectasias

surrounded by skin pallor.

anesthesia that is needed for treatment in older children.6,7

The most common adverse effects of treatment are tran-
sient purpura, localized edema, crusting, and blistering,
but long-term complications such as pigmentary changes
and scarring are rare.8
A nevus simplex is a transient capillary malformation.
INFANTILE HEMANGIOMAS
Infantile hemangiomas are the most common benign vas-
soft tissue hypertrophy that involves underlying struc- cular tumor in children, occurring in up to 5% of infants
tures, such as gingiva. Treatment can reduce the visibil- and 10% of premature infants. They are more common in
ity of lesions and associated psychological morbidity. A infants with low birth weight, female sex, twin gestation,
pulsed dye laser selectively destructs the involved capil- and premature birth.9,10 Clinical presentation at birth varies
laries. Treatment with pulsed dye laser is typically more from bright strawberry-red lesions to pale patches or telan-
effective in children than adults due to differences in skin giectasias surrounded by pallor 11 (Figure 2). Hemangiomas
thickness and capillary size.5 Treatment in infants younger proliferate rapidly between one and three months of age
than one year is effective and avoids the need for general and typically stop growing by five months of age. Involution

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 NEWBORN SKIN: BIRTHMARKS

starts by 12 months of age and lesions disappear by four gestational age or in older infants with poor social support
years of age in 80% of children.10 or cardiovascular or respiratory comorbidities.10 Adverse
Most hemangiomas are benign and do not require treat- effects of propranolol include changes in sleep, acrocyanosis,
ment. High-risk features are those that increase potential bronchial irritation, gastrointestinal symptoms, hypoten-
for life-threatening complications, functional impairment, sion, bradycardia, or hypoglycemia.9,10 Propranolol adminis-
ulceration, underlying abnormalities, permanent scarring, tration with or after feeding and withholding the medication
or alteration of anatomic landmarks. Hemangiomas on during times of reduced intake or vomiting can lower the
the face or anterior neck can be associated with functional risk of treatment-associated hypoglycemia. Systemic or
impairment of feeding or vision or, if they are associated intralesional corticosteroids and topical timolol are alterna-
with airway hemangiomas, life-threatening complications. tives to propranolol.10
The presence of five or more hemangiomas increases the
likelihood of hepatic hemangioma, which rarely can lead to Melanocytic Lesions
high-output heart failure. Hemangiomas in the perineum, Melanocytic or pigmented lesions are caused by displaced
gluteal cleft, or lumbosacral area can be associated with melanocytes, such as those in dermal melanocytosis, or
occult spinal dysraphism or LUMBAR (lower body heman- atypical growth of melanocytes, such as those in congenital
gioma, urogenital abnormalities/ulceration, myelopathy, melanocytic nevi.
bony deformities, anorectal malformations/arterial anom-
alies, and rectal anomalies) syndrome. Hemangiomas with
a diameter greater than 5 cm that involve the face or scalp
can be an indicator of PHACE (posterior fossa anomalies, FIGURE 3
hemangioma, arterial anomalies, cardiac anomalies, and eye
anomalies) syndrome. Hemangiomas located over the axilla,
much of the face, or diaper area have the potential for per-
manent scarring or alteration of key anatomic landmarks.
Urgent referral to a hemangioma specialist is recommended
if any high-risk features are present.10 The Infantile Heman-
gioma Referral Score screening tool (https://​w ww.ihscoring.
com) reliably identifies infantile hemangiomas that require
referral, can be used quickly by primary care physicians, and
has good interrater reliability between expert and nonex-
pert users.12,13
Imaging should be performed if there is concern for
associated anatomic abnormalities. Abdominal Doppler
ultrasonography is indicated in children with five or more
cutaneous hemangiomas or if there is concern for LUMBAR
syndrome. Infants should be evaluated for occult spinal
dysraphism if hemangiomas are present in the perineum,
gluteal cleft, or lumbosacral area. Magnetic resonance angi-
ography of the head and neck should be performed if there is
concern for PHACE syndrome.10
Success rates are significantly higher if treatment is started
before 10 weeks of age, and current guidelines recommend
starting by one month of age.10,14 Systemic beta blockers
are the treatment of choice, specifically propranolol, 2 to
3 mg per kg per day.10 This is supported by moderate-qual-
ity evidence, with a number needed to treat of 2 vs. placebo
for clearance of hemangiomas.15 Propranolol is superior to
systemic atenolol in the rate of complete response;​how- Dermal melanocytosis is characterized by benign,
ever, propranolol is associated with significantly more hyperpigmented macules primarily found in the lum-
adverse effects.16 Treatment should be initiated in the inpa- bosacral and gluteal regions.
tient setting for infants younger than five weeks’ corrected

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 FIGURE 4 TABLE 1

Evaluation of Cutaneous Markers of Occult

Spinal Dysraphism
Lumbosacral skin lesion Suggested evaluation

Atypical dimple* Ultrasonography

Any one of the following:​ Ultrasonography or mag-

Caudal appendage netic resonance imaging

Hemangioma
Hypertrichosis
Subcutaneous mass
(e.g., lipoma)
Congenital melanocytic nevus on the lower Any two skin findings Ultrasonography or mag-
extremity. netic resonance imaging

*—An atypical dimple is larger than 5 mm or located more than

25 mm from the anus. Deep dimples, multiple dimples, or those
that are located cranially to the gluteal crease or outside the mid-
line would also be included in this category.
FIGURE 5
Information from references 20, 21, and 23-25.

nevi should be clearly documented during physical exam-

ination. Lesions tend to increase in size as the child ages, and
the rate of increase depends on the location. Lesions on the
head will likely double in size, whereas those on the trunk
and extremities will likely triple in size.19
The primary concern with a congenital melanocytic nevus
is the risk of melanoma. Lifetime risk of melanoma in any
congenital melanocytic nevus is up to 2.2%;​however, risk
increases with projected adult size. Solitary lesions with a
Cutaneous stigmata of occult spinal dysraphism. This projected size of less than 20 cm may be observed, with refer-
newborn has two simple dimples and a small, palpa- ral for any change in color, texture, size, bleeding, or pain.
ble, subcutaneous mass below the more caudal dim- Immediate dermatologist referral is recommended for a pro-
ple. There is also an unrelated dermal melanocytosis. jected size of greater than 20 cm or with multiple congenital
melanocytic nevi.18 Historically, surgical removal has been
recommended; however, removal does not eliminate the risk
DERMAL MELANOCYTOSIS of melanoma.18-20
Dermal melanocytosis is a benign, hyperpigmented mac-
ule primarily found in the lumbosacral and gluteal regions Markers of Spinal Dysraphism
(Figure 3). Single or multiple macules that are blueish gray Occult spinal dysraphisms are skin-covered developmental
in color may be present and should be documented during abnormalities of the nervous system. Early recognition of
physical examination. No treatment is required. Parents can skin markers of occult spinal dysraphisms and evaluation
be reassured that dermal melanocytosis will fade by two and surgical correction in affected newborns can prevent
years of age and may disappear by puberty.17 irreversible neurologic damage before neurologic impair-
ment is clinically apparent.21 Most cases of occult spinal
CONGENITAL MELANOCYTIC NEVI dysraphisms are associated with midline lumbosacral
Congenital melanocytic nevi are pigmented lesions that skin abnormalities such as atypical dimples, hypertricho-
affect up to 1 out of 17 newborns. Lesions vary in size from sis, capillary hemangiomas, subcutaneous masses, includ-
a few millimeters to several centimeters, may be flat or pal- ing lipoma, or a caudal appendage21 (Figure 5). Features of
pable, and most commonly occur on the trunk, extremities, atypical sacral dimples include dimples that are larger than
or face. The most common locations are the lower back and 5 mm, located more than 25 mm from the anus or off the
thigh area. Color may be uniform or variable within the midline, or with no visible skin at the base.21,22 A solitary
same lesion but is predominantly shades of black or brown18 dimple is a low-risk finding.22 Lumbosacral skin findings
(Figure 4). Locations and sizes of congenital melanocytic other than simple, solitary dimples, especially two or more

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 NEWBORN SKIN: BIRTHMARKS

skin findings, suggest an increased risk of occult spinal 4. Sabeti S, Ball KL, Burkhart C, et al. Consensus statement for the man-
agement and treatment of port-wine birthmarks in Sturge-Weber syn-
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can be used to evaluate newborns with markers of occult spi- port-wine birthmarks. Pediatr Dermatol. 2021;​38(2):​351-358.
nal dysraphisms (Table 1).20,21,23-25 Although ultrasonography is 6. Chapas AM, Eickhorst K, Geronemus RG. Efficacy of early treatment of
facial port wine stains in newborns:​a review of 49 cases. Lasers Surg
more difficult to obtain in newborns after three months of age Med. 2007;​39(7):​563-568.
due to ossification of the vertebral bodies, MRI requires the 7. Jeon H, Bernstein LJ, Belkin DA, et al. Pulsed dye laser treatment of
use of sedation.21,22 Ultrasonography is cost-effective for initial port-wine stains in infancy without the need for general anesthesia
[published correction appears in JAMA Dermatol. 2019;​155(4):​504].
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ysis. Lasers Med Sci. 2023;​39(1):​16.
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9. Püttgen KB. Diagnosis and management of infantile hemangiomas.
than dimples that infer a higher risk of a surgical lesion, evalu- Pediatr Clin North Am. 2014;​61(2):​383-402.
ation with MRI may be a more time efficient and cost-effective 10. Krowchuk DP, Frieden IJ, Mancini AJ, et al.;​Subcommittee on the
approach;​however, in resource-limited settings, ultrasonog- Management of Infantile Hemangiomas. Clinical practice guideline
for the management of infantile hemangiomas. Pediatrics. 2019;​143(1):​
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ings or before imaging if there is discharge from any dimple.21 Buonocore G, Bracci R, Weindling M, eds. Neonatology. A Practical
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borns to be photographed for this article. gold standard for treatment of infantile hemangioma? A systematic
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Address correspondence to Katherine A. M. Snyder, MD,
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Mosaic Community Health, 500 NE A St. Ste. 100, Madras, Rev. 2011;​32(3):​109-113.
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