How can you remember which types of cells are found in scrapings from erythema toxicum neonatorum vs. transient neonatal pustular melanosis ?

Erythema toxicum neonatorum = Eos

Neonatal pustular melanosis = PMNs

If an older child has a rash that looks like what you see in erythema toxicum neonatorum, what would you probably think it was?

Flea bites (helps you remember the appearance)

Is it possible to miss the vesicle phase of transient neonatal pustular melanosis, even if you have been examining the child from birth?

Yes – sometimes the disorder starts in utero(!) so the vesicle phase is already over when the baby is born

How often does aplasia cutis congenita occur as a single lesion?

70 %

Aplasia cutis congenita can occur anywhere on the body. Where are the lesions most often found?

80 % are scalp lesions – usually at the vertex (highest point on the scalp)

If you identify a lesion of aplasia cutis congenita, how should you treat it?

Usually heals on its own

If a lesion of aplasia cutis congenita has already healed on its own, how might you still notice on physical exam that the lesion was previously there?

No hair in a spot on the scalp – The lesion is deep enough that, even when it heals, there are no skin appendages present (glands, hair follicles, and hair)

If you see lesions on the extremities that look like cigarette burns, what infectious agent could be the culprit?

Crusted impetigo – due to GABS or staph

What are the only effective topical agents for impetigo ?

Mupirocin (low cost, and good for limited disease), OR

Retapamulin (newer agent)

Why might you want to culture impetigo?

Increasing incidence of MRSA

What is usually the best choice for treating impetigo, if an oral agent will be used?

Oral cephalexin

Do you need to worry about your patient developing rheumatic fever (or PANDAS – the neuropsych complications of strep infection), if he or she has developed impetigo?

No – skin infections don’t do that

(throat infections do)

(but post-strep glomerulonephritis is still possible)

After infection with HHV-6, how long will the patient excrete the virus?

Lifetime – it’s one of the herpes family!

(HHV-6 causes roseola)

In which patients is the rash of roseola sometimes difficult to detect?

Dark skinned

If HHV-6 infection occurs in an immunocompromised host, the infection can be severe. What treatment is available?

Ganciclovir

What organism is associated with neonatal acne-like pustulosis (also called neonatal cephalic pustulosis)?

Malassezia

Neonatal acne-like pustulosis is clinically different from neonatal acne in what main aspect?

No comedones

At what point in life do port-wine stains become nodular and hypertrophied?

20s–40s

Pulsed-dye laser treatment is a good way to treat port-wine stains, although multiple treatments are usually required. When should laser treatment of port-wine stains ideally be attempted?

Before school age –

To minimize psychosocial impact

(and definitely before the lesions worsen in adulthood)

If you treat a port-wine stain with pulsed dye laser, what must you warn the patient & family about?

They sometimes recur

What is the new name for a cavernous hemangioma ?

“Deep” infantile hemangioma

(“cavernous hemangioma” is no longer used in most literature)

There are two older terms for superficial infantile hemangioma. What are they?

Capillary hemangioma

  &

Strawberry hemangioma

In general, are significantly sized hemangiomas of the genitals treated?

Yes – for both cosmetic and functional reasons

When hemangiomas occur in internal organs, which organs are most often affected?

 (top 3)

1. Liver

2. GI

3. Brain

(in that order)

Avoiding which foods is usually a good idea for children with atopic dermatitis?

Cow’s milk

Eggs

Wheat

Nuts

Can psoriasis have pustules?

Yes – there is a special type that has a lot of crusting and pus (called pustular psoriasis)

What is the most common systemic complication of psoriasis?

Psoriatic arthritis

After a herald patch appears, how long do you expect it to be before the whole rash of pityriasis rosea arrives?

A few days to a few weeks

(not more than 2 weeks)

Which age group usually develops pityriasis rosea?

Adolescent/young adult

How often do patients with pityriasis have a recurrence after the first episode has cleared?

Rare

How long does the rash of pityriasis last?

1–2 months!

(Let’s hope it’s not the type that itches!)

There are several types of ichthyosis. The most common one is ichthyosis vulgaris . Which part of the skin is spared in ichthyosis vulgaris?

The flexures

What is the course of ichthyosis vulgaris, as related to age?

Appears after 3 months old, & improves with age

How is X-linked ichthyosis different from ichthyosis vulgaris, in terms of its course?

Appears before 3 months old, & worsens with age

(No, it doesn’t worsen with sex!)

X-linked ichthyosis has several effects on other organs. Which problem is seen in both affected males and carrier females?

Corneal opacities

Males affected by X-linked ichthyosis often have what other (minor) congenital anomaly?

Cryptorchidism

What are the two types of contact dermatitis?

Irritant & allergic

How are the two types of contact dermatitis different?

Allergic requires previous exposure, and only certain people will develop it

Irritant does not require prior exposure, and affects everyone exposed

Which medication causes allergic contact dermatitis most often?

Neomycin

(topical ointment)

If a patient has been exposed to poison ivy, or something similar (an urushiol), can the dermatitis be spread by the fluid from the bers?

No

Can the contact dermatitis of poison ivy be spread by itching the area, then touching another area?

Sometimes – if the exposure was recent, the fat-soluble urushiol will still be in or on the skin – itching can lodge some under the nails and allow spread to another site

When treating a skin staph aureus infection how concerned should you be that it may not be sensitive to erythromycin?

Very – at least 1/3 are not

Which organism usually causes perianal cellulitis ?

Group A streptococcus

What are the symptoms in kids with perianal cellulitis?

Perianal irritation & persistent red rash

How is perianal cellulitis usually treated?

Systemic antibiotics

(PCN or erythromycin)

Perianal cellulitis is often misdiagnosed as what two other common problems?

Candidiasis or

Perianal fissure

What clinical clue can point you to a diagnosis of staph scalded skin syndrome?

Young child who doesn’t want to be held

(skin is very tender)

We always hear about the sunburn-like rash and hypotension of toxic shock syndrome. Are other body systems also involved?

Yes, definitely

(renal, hepatic, thrombocytopenia, CNS)

What is the special name for herpes infection/recurrence in unusual locations, sometimes experienced by wrestlers?

Herpes gladiatorum

For oral herpes lesions, which over-the-counter medication has good efficacy when applied every 3 h?

Docosanol

(trade name Abreva™)

Can herpetic whitlow sometimes appear to be a much bigger infection?

Yes – there are often swollen joints nearby, and sometimes red streaks/lymphangitis

Are chicken pox patients infectious before they get the rash?

Yes – at least 1 day before the rash

If a patient has been immunized for varicella , can he or she still get a zoster eruption (shingles)?

Yes – the live virus in the vaccine can cause it

Is zoster ber fluid infectious?

Yes – will cause chicken pox in vulnerable patients

If a patient has the rash of erythema infectiosum, and it seems to come and go, is the infection still likely to be erythema infectiosum?

Yes, the rash often comes & goes

If your patient has something that looks a lot like hand-foot-mouth disease, but there is no mouth involvement, what is it?

Papular – pruritic gloves and socks syndrome

Which virus typically causes “papular-pruritic gloves & socks syndrome?”

Parvovirus B19

(also seen with Coxsackie viruses, and some others)

Why are preemies at special risk for skin troubles?

Their skin is structurally different –

The stratum corneum is not mature

What is the importance of the stratum corneum, and what is it?

• It is the topmost, tough layer of the skin

• Creates a barrier between us & outside!

When, in terms of gestational age, does the stratum corneum mature?

About 33 weeks

What is the easiest way to remember what the impact of immature skin will be for the preemie?

 (Mnemonic & 5 consequences)

Mnemonic: Think of them like a burn patient

1. Lose heat

2. Lose fluids

3. Lose energy

4. Increased infection rate

5. Increased med absorption for topical meds

What very unusual cutaneous disease are preemies at special risk to develop?

Invasive

(& then disseminated)

fungal disease

In addition to preemies, what other patient group develops invasive fungal skin infections?

Severely immunocompromised (especially T cell dysfunction)

How do you identify invasive cutaneous fungal infections ?

Well-defined “punched-out” appearance

  Or

Black eschar

(in some cases, they may be more subtle, with subcutaneous nodules and plaques, but these presentations would usually require a specia’s assistance to diagnose)

Which two fungi are especially likely to cause invasive cutaneous disease, in general?

Aspergillus

  &

Rhizopus

Amongst premature and low-birth-weight infants, in particular, what fungi cause invasive cutaneous disease?

Candida

Aspergillus &

Less commonly Trichosporon and Curvularia

When should you suspect a cutaneous fungal infection in a preemie?

Anytime there is an ulceration (of the skin)

What causes the “miliaria” sometimes seen on the skin of infants & young children?

Obstruction of the sweat duct – eccrine gland

(usually by moist stratum corneum cells)

What is miliaria crystallina vs. rubra ?

• Crystallina is very superficial obstruction, & causes only tiny vesicles

• Rubra is a little deeper, & causes 1–3 mm slightly red papules

If a child develops a rash in a hot environment, but appears to be well , and the contents of the papules/papulopustules show neutrophils but no bacteria , what have you got?

Miliaria rubra

Where can miliaria rubra occur?

Almost anywhere –

Including dorsal hands

(not on palms or soles, though)

If you are considering a diagnosis of miliaria rubra for a young child, what test should you do?

KOH prep to rule out candidiasis

(can look very similar)

A 2-day-old infant born at term develops blotches of red on the skin with overlying pustules. The infant is well appearing. What will a Wright stain of the pustule likely show, and what is the disorder?

• Eosinophils and nothing else

• Erythema toxicum

What is the natural course of erythema toxicum?

Spontaneous resolution by age 1 week

How do you recognize eosinophils on micro?

Bright red granules

  &

“Bilobed” nuclei

Which babies are most likely to develop erythema toxicum?

Term babies –

Preemies almost never do

What is the difference between infantile & neonatal acne?

(2)

1. Time of development (neonatal is in first month)

2. Infantile has comedones, neonatal does not

How does neonatal acne present?

Small papules & pustules in the first month of life

What is the natural course of neonatal acne ?

Spontaneously resolves in a few weeks or less

Should neonatal acne be treated?

No –

Wait for resolution

Why does neonatal acne occur?

Androgen stimulation of sebaceous glands

(etiology of infantile unclear)

What types of skin problems are seen with infantile acne?

Papules/pustules

Comedones (open & closed)

Nodules sometimes

What are the common terms for open & closed comedones?

Open = “Black head”

Closed = “White head”

What is the usual course of infantile acne?

Spontaneous resolution after months – median time is about 18 months

Should infantile acne be treated?

If there are nodules – yes

(risk of scarring)

When infantile acne is treated, how is it treated?

Retinoids (topical)

Benzoyl peroxide

Erythromycin (oral or topical)

Which ethnic groups commonly have “Mongolian spots ?”

Asian

African-American

Native American

Where are Mongolian spots usually found?

Lower back & buttocks

(they fade in time)

What is the new name for Mongolian spots?

Dermal melanocytosis

Transient neonatal pustular melanosis refers to what infant dermatological problem?

Superficial pustules →

Superficial erosions →

Hyperpigmented macules

(Hence, the name melanosis!)

How can you differentiate erythema toxicum from transient neonatal pustular melanosis, clinically?

1. E. toxicum has pustules on a reddened macule – melanosis just has pustules

2. E. toxicum doesn’t hyperpigment!

How can you differentiate erythema toxicum from transient neonatal pustular melanosis , in terms of the lab findings?

• Pustule scrapings of E. toxicum → eosinophils

• Pustule scrapings of melanosis → neutrophils

(neither one should have any bacteria or fungus)

Which stage of transient neonatal pustular melanosis are you most likely to see, and why?

The hyperpigmented macules –

The pustules rupture very easily, so you often don’t see them,

The hyperpigmentation lasts for months

What is the most common area of the body for bullous impetigo in infants?

Diaper area

What does bullous impetigo look like?

Large bers (bullae) –

One or more

  Or

Superficial ulcer with collarette if they’ve ruptured

How can you identify ringworm infection?

• Growing border

• Well demarcated

• Central clearing

Does ringworm infection have pustules?

Not usually,

but it can

How can you tell the difference between a tinea infection (ringworm), and nummular eczema (eczema that occur in round, multiple, patches)?

Eczema is itchier

  &

Has more dry skin

If a child has many spots of ringworm, what does that tell you about how he or she acquired the infection?

Usually came from a pet if it’s that bad

Which patients are at increased risk of ringworm infection, although their immune systems are essentially normal?

Atopic patients

Clustered vesicles on an erythematous base is usually what skin infection?

Herpes simplex

If you see clustered vesicles on an erythematous base on a neonate, but you aren’t certain of the diagnosis, what should you do?

 (3 things)

Send serology (IgM for HSV)

Send a viral culture (from the site)

Start empiric IV acyclovir

What is the other name for subcutaneous fat necrosis?

Panniculitis

(Remember that a pile of fat or fat roll is called a “pannus”)

(but other forms of panniculitis also exist, such as those associated with lupus, erythema nodosum, etc.)

Which infants are at special risk of developing subcutaneous fat necrosis?

 (3 groups)

Those with:

1. Trauma

(including birth trauma)

2. Perinatal asphyxia

3. Hypothermia

What do subcutaneous fat necrosis areas look like on physical exam?

Ill-defined, erythematous plaques, indurated

(Can feel them better than you can see them)

Where is panniculitis most common in young infants?

 (4 areas)

Back

Buttocks

Legs

Cheeks

Where is panniculitis most common in older children?

Cheeks –

Due to cold exposure

(including popsicles!)

What is the course for panniculitis?

Spontaneous resolution

For a young infant, or a child with large amounts of panniculitis, what life-threatening complication sometimes develops?

Hypercalcemia!

(Significant & life-threatening levels can be seen!)

What is neonatal lupus erythematosus ?

Development of some aspects of SLE in a newborn due to transfer of maternal autoantibodies

What proportion of mothers who have neonatal lupus erythematosus babies have a history of lupus, themselves?

½ (!)

(Don’t assume the Mom has to have a history!)

What is the prognosis for the mother if her baby has neonatal lupus, and she has no symptoms?

Increased risk of developing lupus –

But not a certainty

How does neonatal lupus present, in general terms?

70 % skin findings

65 % cardiac

>50 % hepatobiliary

What are the usual skin findings of neonatal lupus?

• Usually, erythematous scaly lesions – can be widespread but most often seen on the scalp and face (can be worsened by sun exposure)

• Most prominent around eyes (can be hypopigmented, also)

What is the usual course for neonatal lupus erythematosus?

Spontaneous resolution of skin lesions by 6 months

(as maternal antibodies disappear)

(liver & hematological abnormalities also resolve spontaneously – heart block, if present, is permanent)

If a neonate presents in third-degree heart block, what is the most common cause for the problem?

NLE

(Neonatal lupus erythematosus)

Third degree means no relationship between atrial and ventricular beats

Do neonatal lupus patients typically have other systems involved – aside from heart & skin?

No –

But 10–20 % have (cholestatic) hepatitis & thrombocytopenia

As neonatal lupus babies get a little older, what do you often find on the skin, in addition to the raccoon eye rash?

Annular scaly lesions

(annular = coin shaped)

What is the single best test to diagnose neonatal lupus erythematosus?

“Ro” antibody –

Test Mom & baby

(It is almost always positive in NLE)

What does a “collodion baby ” look like?

Like the baby has an outer wrapping of kind of tight parchment

Although some collodion babies go on to have normal skin, most have what general category of underlying skin disorder?

Ichthyosis

  Or

“Ichthyosiform” erythroderma

Because the outer surface of the skin is unusually tight, collodion babies are at risk to develop what problems at the eyes & mouth?

Eversion of eyelids & lips (due to tension)

(In the eyelid, this is called “ectropion,” while on the lips it is called “eclabium”)

Aside from problems with eyes or mouth, collodion babies are also at risk for what other problems?

Same as a preemie –

The skin barrier is not intact

(infection, fluid & heat loss, increased energy requirement)

Infants who have an impaired or missing stratum corneum (preemies, collodion babies, etc.) need what three interventions?

1. High humidity

2. Bland emollients

(e.g., petroleum jelly)

3. Fluid/electrolyte

monitoring

Infants with impaired strata corneum are most likely to develop what electrolyte problem?

Hypernatremia

(due to dehydration)

Collodion babies are at special risk from what medication-related issue?

Increased absorption

(especially of urea & acid-based agents sometimes used to improve their skin)

What is a “blueberry muffin” baby ?

A baby with dark, raised spots on the skin –

Due to extramedullary hematopoiesis

Where in the skin is the extramedullary hematopoiesis happening, for blueberry muffin babies?

In the dermis

There are a number of reasons for blueberry muffin babies – what is the most common?

Congenital CMV

What is the general idea behind blueberry muffin babies – in other words, what is the cause in general terms?

1. Anything that drops the crit low enough during fetal life can cause blueberry muffin baby

2. Viruses

In addition to CMV, give examples of other infectious diseases that might cause blueberry muffin babies.

(3)

1. Rubella

2. Parvovirus B19

3. Coxsackie

What are some examples of low hemtocrit situations that might produce blueberry muffin baby?

 (4 important examples)

1. Twin-twin transfusion (for the losing twin)

2. Congenital leukemia

3. Blood grp/Rh incompatibility

4. Marrow infiltrating cancers

What does a baby with neonatal erythroderma look like?

Red, scaly baby

Is neonatal erythroderma a worrisome condition?

Yes –

It’s not difficult to manage, but often indicates other significant disorders are present

Erythroderma usually results from what three possible problems?

 (3 groups)

1. Infection (candida or staph scalded skin)

2. Immunodeficiency (SCIDs, GVH, etc.)

3. Ichthyosiform disorders

A skin defect on the head is often an isolated defect, although it is also associated with trisomy 13. What characteristics should make you worry that the defect communicates inside the skull?

• It is midline

• It has a “hair collar” around it

What is a dermoid cyst of the skin?

A congenital tumor in the subQ tissue –

with dermal & epidermal type cells

Where do dermoid cysts of the skin usually develop?

Embryonic fusion lines

(especially the anterior fontanelle, upper lateral forehead, & submental area)

Submental = under the chin

Which dermoid cysts of the skin are most likely to produce complications?

Those with sinus tracts connecting them to the surface

(tuft of hair sometimes at surface)

(main complication is infection)

What is the recommended management for dermoid cysts?

Elective excision

(they sometimes turn malignant!)

A scalp nodule in the midline, present at birth, suggests ___________?

Cranial dysraphism

(meaning underlying lack of closure with possible neural abnormalities)

Do dermoid cysts of the face or scalp frequently have intracranial connections?

No – only if they are midline (25 %)

What does a “hair collar” look like?

Dark, coarse, & longer hair surrounding a current or healed defect (suggests cranial dysraphism)

When is ultrasound a good way to screen for spinal dysraphism ?

• Infants <6 months

• Low level of suspicion

If ultrasound is not appropriate, what diagnostic should you use to evaluate for spinal dysraphism?

MRI

If an infant has spinal dysraphism, what do you expect to find on physical exam?

Usually nothing

(can be lower extremity/anal perineal findings, sometimes)

If the gluteal cleft deviates from midline significantly, what is that likely to indicate?

Underlying dysraphism

What type of vascular changes often signal underlying dysraphism?

Hemangiomas & vascular stains

(= darkened areas of skin due to dense capillary areas)

Spinal dysraphism typically occurs in what portion of the spine?

Lower midline

Technically, are hemangiomas malformations or tumors?

Tumors

What is the most common tumor of infancy?

Hemangiomas

If a child has a hemangioma, will it be present at birth?

Sometimes –

but they can appear up to 1–2 months after birth

Which infants are most likely to have hemangiomas?

 (3 risk factors)

Preemie girls with h/o chorionic villus sampling

How common are hemangiomas in 1-year-old children?

10 %!

(Data for 1-year-olds based on Caucasian infants, due to lack of data for other ethnicities. Percentage with hemangiomas at birth is 1–2 % in various ethnicities)

Infantile hemangiomas come in two flavors – what are they?

Superficial & deep

(“combined” is also a possibility)

How can you identify a superficial hemangioma?

Very red

Very well defined

(+ superficial is most common, so if in doubt, guess superficial!)

How does a deep hemangioma look different from a superficial hemangioma?

Deep are:

1. Either skin colored or violaceous

2. Raised

3. Not well defined

If a hemangioma is “mixed” or “combined” (same thing), what will it look like?

Ill-defined raised area

  +

Some deep-red sharply demarcated areas

What is the most important complication to develop from liver hemangiomas?

High-output CHF!!!

Hemangiomas follow a characteristic pattern of growth, and they can be identified by it. What is the pattern?

Proliferation

(weeks up to about a year)

Stability

Involution

(starts around 1 year, and takes years to finish)

What is the first sign of involution for a superficial hemangioma?

Loss of the bright color –

First central,

then peripheral

Do deep hemangiomas involute faster or slower than the superficial ones?

Slower

(they soften & flatten)

After involution, does a hemangioma completely disappear, or does it leave residual changes?

Either –

Often some residual changes such as surplus skin or fibrofatty changes in the area

By age 5, what percentage of hemangiomas have involuted?

>50 %

(50 at 5)

By age 9, what percentage of hemangiomas have involuted?

90 %

(90 at 9)

Is bleeding from a hemangioma life threatening?

No –

(although it can still be pretty annoying!)

A relatively common complication of hemangioma occurs mainly when the tumor is on the lip, nose, or perineum. What is the complication?

Ulceration

Which hemangiomas are most likely to ulcerate, in terms of their growth pattern?

Large & rapidly growing

What are the main concerns when a hemangioma ulcerates?

Infection

Scarring

Bleeding &

Pain

How are hemangioma ulcerations managed?

• Vaseline or zinc oxide barrier protection

• Occlusive dressing

• Culture & antibiotics as needed

What are the main signs of infection in an ulcerated hemangioma?

Poor healing, or exudates (of course!)

What unrelated problem can look similar to a nasal hemangioma?

Encephalocele!

(yikes!)

The bluish hue and raised nature of the dacryocystocele can look similar to which type of hemangioma?

Deep hemangioma

(dacryocystoceles are accumulations between the eye & nose, related to the tear duct)

Why should an infant with a periorbital hemangioma have close monitoring from ophthalmology?

 (2 reasons)

1. Possible obstruction of the visual axis and development of astigmatism

2. May have retroorbital hemangioma that you can’t see (proliferative phase can be a big deal!)

Why might hemangiomas near the mouth, or on the neck, be concerning?

(especially if bilateral)

Possibility of airway compromise (from subglottic hemangioma)

When hemangiomas are in very dangerous locations, or creating very serious complications, what is usually done to manage them?

 (2 options)

Glucocorticoids or propranolol

(Propranolol appears to decrease hemangioma growth, as well as constrict the existing vessels)

How common are ocular complications with periorbital hemangiomas?

Very common – 80 %

How might a retroorbital hemangioma present on exam?

Proptosis

How are the steroids delivered when they are used to treat a problematic hemangioma?

Intralesional and/or systemic, depending on the lesion

What is the special name for hemangiomas that develop near the airway (chin, mandible, and upper neck)?

“Beard” hemangiomas

How do beard hemangiomas present, if they are threatening the airway?

The usual ways

(Stridor, cough, hoarseness, noisy breathing, cyanosis)

If a baby presents to your practice at age 15 months with a beard hemangioma, how urgently should you be concerned about the airway?

Not concerned –

The proliferative phase is over

What percentage of kids with extensive beard hemangiomas (4 out of 5 beard regions involved) will have airway involvement?

60 %

Lumbosacral hemangiomas are highly associated with disorder of what nearby structure?

Spinal cord –

Tethered cord

What are the main complications of ear hemangiomas?

Disfiguring

  &

Speech delay due to obstruction of the EAM

Lumbosacral hemangiomas are significantly associated with anomalies of what somewhat distant structure?

Kidneys

Is the dark skin of a port-wine stain a hemangioma?

No –

It is an area of permanent capillary malformation

Any idea what a “segmental cervicofacial” hemangioma is?

A large facial hemangioma that seems to follow an anatomic territory, such as the upper face

Why is it important to know about segmental cervicofacial hemangiomas?

Because they are part of the PHACES syndrome

What are the components of PHACES syndrome?

P osterior fossa malformations

 (like Dandy-Walker)

H emangioma

A rterial anomalies

 (intracerebral)

C ardiac anomalies/Coarc

E ye abnormalities

S ternal defects

If an infant has five cutaneous hemangiomas, what should you consider?

(Or more than five, of course!)

“Diffuse neonatal hemangiomatosis”

(means that hemangiomas may be found in the organs)

Which organs are typically involved in cases of diffuse hemangiomatosis?

Liver (most common)

GI

CNS & eye

When hemangiomas occur in the liver, what special consequences can occur?

Portal hypertension & obstructive jaundice

(both are rare)

Patients with diffuse hemangiomatosis are at increased risk for what very important complication of hemangioma disease?

Congestive

Heart

 Failure

What examinations should be considered for an infant with five or more cutaneous hemangiomas – in addition to a good physical exam?

(4)

1. Liver ultrasound (required)

2. Chest imaging

3. Check stool & urine for blood

4. Eye exam

How useful is laser therapy for hemangiomas?

Medium –

Lasers only penetrate about 1 mm, so it depends on the hemangioma

What is the main management approach to hemangioma?

“Active nonintervention”

(meaning parental guidance & monitoring of the lesion)

When medical intervention for hemangioma is indicated, what is the first-line treatment?

Corticosteroids (PO or IV)

(2–3 mg/kg/day)

  Or

Propranolol (various routes)

(inappropriate in infants with high risk for cerebrovascular incidents)

Which hemangiomas require treatment?

(3)

1. Life or function threat (CHF, airway, vision)

2. Deformity issues (lip, nose, ear) & very large facial ones

3. Ulcerations

Why is Kasabach–Merritt syndrome important to know about?

Because this vascular tumor often causes a life-threatening consumptive coagulopathy

What is Kasabach–Merritt syndrome due to?

A rapidly growing congenital vascular tumor

(NOT a hemangioma, but it was previously thought to be one!)

Where on the body do the tumors that cause Kasabach–Merritt syndrome occur?

Superficial/skin

  Or

Deep/visceral

(just about anywhere)

What is the “syndrome” part of Kasabach–Merritt syndrome?

 (3 components)

The vascular tumor

  +

Thrombocytopenia due to platelet trapping

  +

Consumptive coagulopathy

What is the prognosis for Kasabach–Merritt syndrome patients?

Not good –

High mortality

What kinds of tumors are responsible for Kasabach–Merritt syndrome?

(2)

Tufted angioma

  &

Kaposiform hemangioendothelioma

How is Kasabach–Merritt syndrome (or the vascular tumor that could cause it) treated?

Many techniques, depending on size & location:

1. Systemic corticosteroids

2. Alpha interferon & vincristine

3. Surgical excision/arterial embolization

4. Radiotherapy

When Kasabach–Merritt syndrome is recognized, how urgent is it to begin treatment?

Emergency!

Vascular malformations are not tumorous growths, but rather errors in the way a structure grew. Vascular malformations occur in what types of vessels?

All types

(arteries, veins, capillaries, lymphatics)

What is a “combined” vascular malformation?

Vein

  +

one of the other vessel types is involved in the malformation

Which type of vascular malformation has the potential for serious blood loss?

Arteriovenous malformation (AVM)

(It is the only type with fast flow/serious pressure)

What is the classic example of a capillary malformation ?

Port-wine stain

(aka nevus flammeus)

What are the common names for a nevus simplex?

“Stork bite”

“Angel kiss”

“Salmon patch”

What is the usual course of a nevus simplex ?

They fade in time

Port-wine stains are most common on what part of the body?

The face

What is the natural course of a port-wine stain?

• They get darker & thicker over time

• They “grow with the child” (continue to cover the same portions of the face as the child ages)

What is a port-wine stain?

Malformation in the upper dermis of “mature” capillaries

“Port-wine stains” have two other names in medicine. What are they?