19 - 103 - NEONATAL DERMATOLOGY Flashcards
Results from subcutaneous edema over the presenting part of the head; soft to palpation with ill-defined borders.
a. Cephalhematoma
b. Subgaleal hemorrhage
c. Cerebral contusion
d. Caput succedaneum
D
Results from rupture of the diploic or emissary veins; firm to palpation, respects suture lines
a. Cephalhematoma
b. Subgaleal hemorrhage
c. Cerebral contusion
d. Caput succedaneum
A
Blotchy erythematous patches with a central vesicle are more commonly seen in?
a. Premature infants
b. Term infants
c. Post term infants
d. Any of the above
B
What is the size range of large L2 CMN’s?
a. 30-40cm
b. 20-30cm
c. 10-20cm
d. 40-50cm
A
What imaging modality is recommended for port-wine stains?
a. MRI
b. UTZ if younger than 4 months of age; MRI if over 4 months if age
c. Cranial CT Scan
d. None
D
Characterized by well-defined erythema, white scales on trunk and body; with scaling usually absent in skin folds
a. Psoriasis
b. Diaper Dermatitis
c. Candidiasis
d. Seborrheic Dermatitis
A
Candida from intestinal flora may contaminate and exacerbate diaper dermatitis present for longer than how many day/s?
a. 4 days
b. 3 days
c. 1 day
d. 5 days
B
characteristic clinical finding of hyperimmunoglobulin E syndrome
papulopustular dermatitis of the face and scalp
differential diagnosis when evaluating diaper eruptions
Infantile seborrheic dermatitis, napkin psoriasis, bullous impetigo, acrodermatitis enteropathica, scabies, herpes simplex infections, and Langerhans cell histiocytosis
transient piloerection and induration, which is termed the pseudo-Darier sign is seen in what condition
Congenital smooth muscle hamartomas (CSMHs)
Useful clinical indicator of cutaneous mastocytosis
Tryptase
epidermis begins to develop around how many weeks AOG
**6 weeks **of gestational age as a basal cell layer and a superficial periderm layer
**Stratification of the fetal epidermis **begins at how many weeks AOG
eighth week of gestation
terminal differentiation in the skin appendages occurs how many weeks AOG?
11th and 15th weeks of gestation
dermal–epidermal junction is evident by how many weeks AOG?
8 weeks
collagen fiber formation and organization of the** papillary and reticular dermis** occurs by
15 weeks of gestation
**Hair follicles **begin to develop around what week AOG?
14th week of gestation
elastic fibers are noted at what week AOG?
22 to 24 weeks of gestation
lanugo hair production begins around what week AOG?
18th week of gestation and is complete by the 28th week of gestation
Sebaceous gland formation begins around how many weeks AOG?
16th week of gestation
vernix caseosa begins to form at what AOG?
36 weeks AOG
T/F
In full-term neonates, after drying of the skin in the first few hours after birth, TEWL is equivalent to that seen in adults,
TRUE
with the exception of some variability because for site and ambient temperature and/or humidity, including the diaper area, where TEWL vales are higher and contribute to impaired barrier function and risk for diaper dermatitis
T/F
In the term and preterm neonate, skin pH is more acidic than that of older infants and adults, and it decreases gradually over the first few weeks of life
FALSE
In the term and preterm neonate, skin pH is more alkaline than that of older infants and adults, and it decreases gradually over the first few weeks of life
functional maturation of the stratum corneum begins around how many weeks AOG?
24 weeks of gestational age
Recommendations for routine skin care in the neonate, as provided by a panel of European experts
- bathing in water 2 to 3 times per week for no more than 5 to 10 minutes with use of a gentle soap-free liquid skin cleanser as opposed to a washcloth;
- application of an emollient after bathing is also recommended
results from **subcutaneous edema **over the presenting part of the head owing to pressure against the cervix and is a common occurrence in newborns
Caput succedaneum
- soft to palpation with** ill-defined borders.**
- Petechiae and ecchymosis also may be noted.
- The edema resolves spontaneously over 7 to 10 days.
Caput succedaneum
- results from rupture of the diploic and/or emissary veins and subsequent subperiosteal collection of blood
- It is associated with birth trauma or the use of vacuum extraction vaginal delivery.
Cephalohematoma
- Areas of hemorrhage respect the suture lines, are **well-delimited, **usually unilateral, and feel firm to palpation
- An underlying skull fracture may be present
- usually resolve without sequelae over several weeks, but calcification may occur
Cephalohematoma
When milia is persistent and/or numerous, they may be seen in association with several rare genetic disorders. Give examples
- oral-facial-digital syndrome
- Bazex-DupréChristol syndrome
- Basan syndrome
- associated with birth trauma and occurs when there is bleeding between the periosteum of the skull and the galea as a result of damage to the** emissary veins**
- bleeding can be catastrophic and result in hypovolemic shock or disseminated intravascular coagulation
Subgaleal hemorrhage
- idiopathic, common skin condition seen in up to 75% of term newborns
- rarely seen in premature infants
- blotchy erythematous patches 1 to 3 cm in diameter with a 1- to 4-mm central vesicle or pustule
- usually begins at** 24 to 48 hours of age**, but delayed eruption as late as 10 days of age has been documented
- benign and clears spontaneously by 2 to 3 weeks of age without residua
ERYTHEMA TOXICUM NEONATORUM
Minute (<1 mm) yellow macules or papules are commonly seen at the opening of pilosebaceous follicles over the nose and central forehead of term newborns
SEBACEOUS GLAND HYPERPLASIA
This is a benign condition thought to occur secondary to maternal androgen stimulation of the sebaceous glands; it typically resolves spontaneously by 4 to 6 months of age.
Erythema toxicum neonatorum tend to spare what areas?
These follicular-based lesions can be located anywhere but tend to spare the** palms and soles.**
milia in the oral cavity
Epstein pearls
A smear of the central vesicle or pustule of erythema toxicum neonatorum contents will reveal what infiltrates?
numerous eosinophils on Wright-stained preparations
A peripheral blood eosinophilia of up to 20% may be associated, particularly in infants, with numerous lesions
idiopathic pustular eruption of the newborn that resolves with hyperpigmented macules
TRANSIENT NEONATAL PUSTULAR MELANOSIS
It is less common than erythema toxicum neonatorum and is more prevalent among newborns with darkly pigmented skin.
TRANSIENT NEONATAL PUSTULAR MELANOSIS
- Lesions are usually present at birth or shortly thereafter, but may appear as late as 3 weeks of age.
- The eruption is characterized by **multiple superficial vesicles and pustules, **with ruptured lesions evident as collarettes of scale
- Hyperpigmented macules are also often present at birth or develop at the sites of resolving pustules or vesicles within several hours of birth or during the first days of life.
- Lesions can occur anywhere but are common on the forehead and mandibular area.
- The palms and soles may be involved.
- pustules usually disappear within 5 to 7 days; residual pigmented macules resolve over several weeks
TRANSIENT NEONATAL PUSTULAR MELANOSIS
Smear of the vesicle or pustule from transient neonatal pustular melanosis contents will reveal a predominance of what infiltrates?
neutrophils with occasional eosinophils on Wright-stained preparations
Miliaria or “heat rash” is a common disorder of what glands?
eccrine glands
typically results from fever or overheating such as may occur when neonates are swaddled
Eccrine gland occlusion by sweat and possibly by the extracellular polysaccharides of Staphylococcus epidermidis leads to the development of minute superficial subcorneal vesicles called?
miliaria crystallina
deeper level of occlusion of eccrine glands within the epidermis with associated inflammation leads to what condition?
miliaria rubra
- manifests as 1- to 3-mm erythematous papules and/or papulopustules
- condition resolves spontaneously once the inciting factors are addressed.
blotchy or lace-like pattern of dusky erythema over the extremities and trunk of neonates that occurs with exposure to cold environments
Mottling
- Virtually all babies demonstrate mottling at some time during the newborn period as a consequence of immature autonomic control of the cutaneous vascular plexus
- this physiologic mottling disappears on rewarming, differentiating it from cutis marmorata telangiectatica congenita and livedo reticularis
- Physiologic mottling resolves spontaneously by 6 months of age.
- when the neonate is placed on one side, an erythematous flush with a sharp demarcation at the midline develops on the dependent side, and the upper half of the body becomes pale
- color change usually subsides within a few seconds of placing the baby in the supine position but may persist for as long as 20 minutes
- Presentation may be localized to a single body area or may be more generalized
Harlequin color change
The exact mechanism of this unusual phenomenon is not known, but it may be a result of immaturity of autonomic vasomotor control
Harlequin color change is seldom seen after 10 days of age, and if noted to be persistent or to develop at an older age, may be a manifestation of an underlying what disorder?
neurologic disorder
Harlequin color change is a rare vascular phenomenon that occurs in what age?
full-term newborns, as well as in low-birthweight and premature infants, and in infants exposed to** hypoxia or systemic treatment with prostaglandins**
Sucking blisters may be present at birth as the result of?
intrauterine sucking
- but are more commonly seen during the first weeks of life
- usually solitary, intact, oval or linear blisters, superficial erosions, or crusts, arising on noninflamed skin on the dorsal aspect of the forearms, wrists, or fingers; a callus may be noted on the upper lip
- resolve within a few days
- if the affected extremity is brought up to the infant’s mouth, the infant will often commence sucking at the site, confirming the diagnosis
most common neonatal acneiform facial eruptions
BENIGN NEONATAL CEPHALIC PUSTULOSIS
Neonatal cephalic pustulosis has been attributed to overgrowth of what organism?
Malassezia spp
Most cases resolve spontaneously, but the eruption can be treated topically with ketoconazole
characterized by blue-gray patches typically located on the lumbosacral or buttock skin of infants
Dermal Melanocytosis (commonly called “Mongolian spots”)
Collections of dermal melanocytes cause these common birthmarks, which are seen in 80% to 90% of infants of color and only 5% of white infants
A large, geographic café-au-lait macule with a “coast-of-Maine” appearance is most likely to be an isolated skin finding but may be a marker of what syndrome?
McCuneAlbright syndrome
Café-au-lait macules are well-demarcated tan macules or patches.
Solitary café-au-lait macules are very common, however, the presence of multiple café-au-lait macules raises the possibility of what condition?
neurofibromatosis type 1
Large and giant CMN are associated with an increased risk of?
Melanoma
- Additionally, individuals with large or giant CMN may be at risk for complications of **neurocutaneous melanocytosis, **particularly if the large or giant CMN has a posterior axial location or if more than 20 satellite nevi are present
- Newborns with more than 2 medium-sized CMN are also at increased risk of neurocutaneous melanocytosis.
- Neurocutaneous melanocytosis results from abnormal melanocytic proliferations in the CNS, and can be complicated by obstructive hydrocephalus and primary CNS melanoma
refers to areas of skin hypopigmentation or hyperpigmentation that are genetically determined by late somatic mosaic genetic mutations in skin cell progenitors
Pigmentary mosaicism (patterned dyschromatosis)
- The dyschromia in pigmentary mosaicism tends not to cross the midline
- In most cases, pigmentary mosaicism is an isolated finding, but the practitioner should maintain high suspicion for associated disease in children with widespread skin dyschromia, associated neurologic, cardiac, or musculoskeletal disease, or in children who are delayed in accomplishing expected developmental milestones
* well-demarcated hypopigmented patch present from birth, which may sometimes become more visible in the first year of life as the background skin pigmentation of the newborn gradually increases
* does not blanch on diascopy
Nevus Depigmentosus
Three or more nevus depigmentosus/hypomelanotic patches in a newborn should prompt evaluation for what possible condition?
tuberous sclerosis complex
- hypopigmented patch that results from** focal vasoconstriction**
- attributed to skin vessel hypersensitivity to catecholamines in the affected areas
- blanches on diascopy
Nevus Anemicus
- represents a superficial vascular ectasia of the capillaries
- It occurs most commonly on the glabella, upper eyelids, and nuchal area, and is colloquially termed “salmon patch,” “stork bite,” or “angel kiss.
Nevus Simplex
Although the majority of nevus simplex birthmarks fade over the first 2 years of life, those on what area of the scalp scalp tend to persist?
Occipital
most common tumors of infancy
Infantile hemangiomas
benign skin hamartoma comprised of numerous sebaceous glands that most often appear as yellow-hued hairless plaque on the head of newborns
Nevus Sebaceus
- Around puberty, a nevus sebaceus may thicken and develop hyperkeratotic, verrucous features.
- Secondary benign neoplasms, including trichoblastoma or syringocystadenoma papilliferum, sometimes develop within a nevus sebaceus, and, uncommonly, basal cell carcinoma has been reported to arise within a nevus sebaceus
Widespread nevus sebaceous skin lesions may be associated with increased risk of abnormalities in the central nervous, eye, or skeletal systems. What is this syndrome?
Schimmelpenning or nevus sebaceus syndrome
The highest risk of occult spinal dysraphism occurs with the presence of what skin lesions?
- 2 or more congenital midline lumbosacral skin lesions or
- if spinal cord dysfunction exists in the presence of 1 lumbar skin lesion
Identify if high, intermediate or low risk for occult spinal dysraphism:
2 or more congenital midline lumbosacral skin lesions
high risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
spinal cord dysfunction exists in the presence of 1 lumbar skin lesion
high risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
lipoma
high risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
tail
high risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
Atypical sacral dimple (deep, farther than 2.5 cm from the anus, size ≥5 mm)
Intermediate risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
dermal sinus
high risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
Unclassified hamartoma
intermediate
Identify if high, intermediate or low risk for occult spinal dysraphism:
Aplasia cutis congenital
intermediate
Identify if high, intermediate or low risk for occult spinal dysraphism:
Port-wine stain
low risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
Deviation of gluteal crease
intermediate
Identify if high, intermediate or low risk for occult spinal dysraphism:
Hypertrichosis (unless large and/or unusual)
low risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
Pigmented nevus
low risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
Mongolian spot
Identify if high, intermediate or low risk for occult spinal dysraphism:
Simple sacral dimple (<5 mm diameter, 2.5 cm or closer to the anus)
low risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
MRI indicated
high risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
Ultrasonography if younger than age 4 months (then MRI if ultrasonography is abnormal), MRI indicated for age older than 4 months, or if sonographic expertise unavailable
intermediate risk
Identify if high, intermediate or low risk for occult spinal dysraphism:
No imaging needed
low risk
composed of heterotopic neural tissue and present as firm, skin-colored to erythematous nodules on the nose
NASAL GLIOMA
congenital anomalies that typically arise along planes of embryonic fusion of the face and scalp
dermoid cyst
formed when the meninges and cerebrospinal fluid herniate through a defect in the calvarium or vertebrae.
meningocele
- midline anomaly that may be associated with a persistent intracranial defect; as such, preoperative imaging is recommended prior to surgical excision and reconstruction
neural tissue of the spinal cord + meninges + cerebrospinal fluid herniate through a defect in the calvarium or vertebrae
myelomeningocele
most common presentation of spina bifida
myelomeningocele
herniation of neural tissue along with the meninges and cerebrospinal fluid through a calvarial defect
ENCEPHALOCELE
Encephaloceles are often seen in association with other craniofacial defects, and neurodevelopmental sequelae may occur.
most common branchial cleft anomaly
Branchial cysts
- majority arise from the second branchial arch and present along the sternocleidomastoid muscle on the lateral aspect of the neck
- definitive treatment for branchial cleft anomalies is surgical excision
congenital neck anomalies that present as a midline mass arising anywhere from the suprasternal notch to the posterior tongue
Thyroglossal duct cysts
- They arise from the developmental remnant of the migrational tract of the thyroid gland and, as such, may be associated with ectopic thyroid tissue.
- Surgical excision is the definitive treatment; preoperative verification of a normal thyroid gland is mandatory.
localized areas of absence of the skin and sometimes the underlying subcutaneous tissues and bone
Aplasia cutis congenita (ACC)
- ACC has no single underlying cause and appears to be the end result of a number of distinct pathologic processes, including a forme fruste of a neural tube defect
- Aplasia cutis is always hairless, and when healed, lesions are usually atrophic scars, although keloidal scarring has been observed.
- Superficial lesions may involve only the epidermis and superficial dermis; in more-severe cases, a full-thickness tissue defect may be present, including involvement of the calvarium.
ring of darker and/or coarser terminal hairs on the scalp, typically surrounding ACC, dermoid cyst, encephalocele, meningocele, or heterotopic brain tissue
hair collar sign
hair collar sign itself is a marker of what?
cranial dysraphism
its presence, like aplasia cutis, mandates careful examination of the infant
vast majority of neonatal HSV cases are caused by
HSV type 2
characteristic clinical finding of hyperimmunoglobulin E syndrome in infancy
papulopustular dermatitis of the face and scalp
management of seborrheic dermatitis in infants
- For mild cases, application of baby oil or mineral oil to the affected areas of the scalp, followed by gentle combing and shampooing to remove scales, is sufficient.
- For more severe involvement, use of a topical antifungal shampoo or cream or a low-potency topical corticosteroid may be necessary.
most common cause of diaper dermatitis
Irritant contact dermatitis
- characterized by erythematous, moist, and sometimes scaly patches favoring the convex surfaces of the genitalia and buttocks.
- The skin folds are classically spared.
- Barrier diaper creams and frequent diaper changes are very helpful to treat this condition.
Candida from intestinal flora may contaminate and exacerbate any type of diaper dermatitis present for longer than how many days?
3 days
second most common cause of diaper dermatitis and is characterized by bright red erythematous, moist papules, patches, and plaques that tend to involve body folds as well as convex surfaces
Candidiasis
Satellite papules and pustules are frequently observed, and oral thrush may be present.
flat-topped, skin-colored papules that develop in the diaper and perianal areas in patients of any age whose skin is chronically exposed to moisture, including children with prolonged urinary or fecal incontinence
Pseudoverrucous papules and nodules
- uncommon, severe diaper dermatitis that is characterized by welldemarcated, punched-out ulcers and erosions
- Prolonged contact with urine and feces under occlusion leads to this condition
Jacquet erosive dermatitis
- uncommon diaper rash characterized by reddish purplish nodules of different sizes (0.5 to 3 cm) occurring on the convexities of the diaper area in 2- to 9-month-old infants
- It arises within preexisting diaper dermatitis.
Granuloma gluteale infantum
- It appears to be an unusual reaction to the usual irritant factors, Candida infection, and, in some cases, topical corticosteroid use in the diaper region
- Treatment consists of avoidance of irritants, use of a barrier ointment, and avoidance of topical corticosteroids.
- Resolution occurs over several months
- smooth pink nodules that appear on the phalanges of infants.
- Most commonly the fingers are involved, and the thumbs and great toes are classically spared
Infantile digital fibromas
benign dermal proliferations of hyperplastic smooth muscle
Congenital smooth muscle hamartomas (CSMHs)
- most often appear as a solitary irregular dermal plaque on the trunk or proximal extremities.
- The overlying skin tends to be skin-toned to slightly hyperpigmented and may have associated hypertrichosis
how do you elicit pseudo-Darier sign?
this is elicited in 80% what condition?
- Brisk rubbing of a CSMH results in transient piloerection and induration
- Congenital smooth muscle hamartomas (CSMHs)
second most common solid tumor of childhood
Neuroblastoma
Skin biopsy of cutaneous lesions of LCH shows a histiocytic infiltrate that stains positive for what?
CD1a and CD107 (Langerin)
how do you elicit Darier sign?
In what condition is this observed?
- Brisk stroking of affected skin will result in mast cell degranulation with a wheal and flare reaction
- cutaneous mastocytosis, urticaria pigmentosa
stains for mast cells
giemsa, c-kit (CD117)
In cases with extensive skin disease or a concerning review of symptoms in cutanenous mastocytosis, what serum marker may be a useful clinical indicator?
serum tryptase
more sensitive indicator of systemic mast cell disease in children when compared to serum tryptase or history of severe mediator symptoms, and that serum tryptase levels tend to decrease over time in children
enlargement of the liver and/or spleen
Incontinentia pigmenti results from mutations in what gene?
nuclear factor κB essential modulator (NEMO) gene
- firm, red to purple subcutaneous nodules or plaques on the back, cheeks, buttocks, arms, and thighs
- lesions usually appear within the first 2 weeks of life and resolve spontaneously over several weeks
- biopsy demonstrates lobular fat necrosis with needle-shaped clefts in lipocytes and mixed inflammation with lymphocytes, macrophages, and giant cells.
Subcutaneous fat necrosis of the newborn
Infants affected by subcutaneous fat necrosis of the newborn are at risk for what serum abnormality?
hypercalcemia
- describes diffuse skin hardening in a sick, premature newborn
- It is thought to be exceedingly uncommon with modern neonatal care.
- The onset is characteristically after 24 hours of age
- Critically ill premature neonates with sepsis, hypoglycemia, acidosis, or other severe metabolic abnormalities are at most risk.
- The skin becomes hard and appears shiny.
- Biopsy shows needle-like crystals within lipocytes but no associated inflammatory infiltrate or fat necrosis, differentiating it from subcutaneous fat necrosis of the newborn
SCLEREMA NEONATORUM
- **persistent, reticulated atrophic violaceous vascular patches **
- sometimes associated with telangiectasias and ulceration
- Most frequently, a lower extremity is involved, but location on the trunk or upper extremity is not uncommon
- Limb asymmetry may be present.
- However, the majority of patients have a good prognosis, with half demonstrating improvement of the mottled appearance over the first 2 years
CUTIS MARMORATA TELANGIECTATICA CONGENITA
