Chapter 35 - Vesiculobullous and Erosive Diseases in the Newborn Flashcards
Occasionally newborns can get herpes zoster. In what clinical scenario might this occur?
In patients born to mothers with a primary varicella infection during pregnancy or up to a few days after delivery
How old does a newborn have to be in order to catch scabies?
Usually 3-4 weeks or older
When does erythema toxicum neonatorum usually present?
Within 24-48 hours, but can be present from birth to two weeks
Which babies are affected by erythema toxicum neonatorum: term infants or preterm infants?
Term infants
Does erythema toxicum neonatorum affect the palms and soles?
No
If the lesions of erythema toxicum neonatorum are scraped and stained with a Wright’s stain, what inflammatory cells are seen under the microscope?
Eosinophils
What percentage of full-term neonates are affected by erythema toxicum neonatorum?
50%
Should erythema toxicum neonatorum be treated?
No; it resolves spontaneously without therapy within 1-2 days
True or false: the skin lesions associated with erythema toxicum neonatorum can be macules, wheals, vesiculopustules, or papules.
True! There are four distinct skin lesions associated with this entity
Are infants with darkly pigmented skin or lightly pigmented skin more commonly affected by transient neonatal pustular melanosis?
Darkly skinned infants; it occurs in 5% of newborns with darkly pigmented skin
Is transient neonatal pustular melanosis predominantly neutrophilic or eosinophilic?
Neutrophilic
Are the palms and soles affected in transient neonatal pustular melanosis?
Yes; there’s widespread involvement, including the palms and soles
When does transient neonatal pustular melanosis typically develop?
Skin lesions are virtually always present at birth
Histologically, where does the pusutle occur in transient neonatal pustular melanosis?
Subcorneal region
Is treatment necessary for transient neonatal pustular melanosis?
No; it’s a benign condition that resolves spontaneously
What are the three variants of miliaria?
Miliaria crystallina, miliaria rubra, and miliaria profunda
True or false: histologically, the inflammation seen in miliaria crystallina occurs around the acrosyringium.
True; obstruction occurs as the eccrine sweat duct passes through the stratum corneum, and sweat collects beneath the stratum corneum
What is the other term for “malphigian layer”?
Spinous layer
What is the synonym for neonatal cephalic pustulosis?
Neonatal acne
When is the typical onset for neonatal cephalic pustulosis?
Within the first 2-3 weeks of life
Are comedones present in neonatal cephalic pustulosis?
No; it’s a non-comedonal pustular eruption
How long does it usually take for neonatal cephalic pustulosis to resolve?
Weeks to months; therapy is not usually necessary
Which infectious organisms have been implicated in neonatal cephalic pustulosis?
M. furfur, M. sympodialis, and M. globosa
Normally no treatment is necessary for neonatal cephalic pusulosis, however, if treatment is desired, what topical medication can help?
A topical imidazole
If a newborn with “neonatal cephalic pustulosis” has acne reminiscent of classic acne vulgaris (e.g. the presence of comedones), what is the condition referred to?
Infantile acne; neonatal cephalic pustulosis only refers to non-comedonal “acne”
What are the two distinct clinical presentations of cutaneous candidiasis in the newborn period? Hint: candidiasis can be due to an infection acquired during delivery (or postnatally), or due to an infection acquired in utero.
Neonatal candidiasis (infection acquired during delivery or postnatally) and congenital candidiasis
What is the treatment of choice for neonatal candidiasis? Topical or systemic therapy?
Topical anti-yeast therapy is preferred, but if the infant is premature, they should be monitored closely, and treated with systemic therapy at the first sign of a systemic infection
True or false: congenital candidiasis usually presents after the first week of life, while neonatal candidiasis presents at birth.
False; the reverse is true. Neonatal candidiasis usually occurs after the first week of life (favoring the diaper area and oral mucosa) while congenital candidiasis is present at birth (diffuse skin involvement, with sparing of the diaper area and oral mucosa)
List two risk factors for congenital candidiasis.
Maternal history of vaginal candidiasis and foreign body in the uterus or cervix (e.g. IUD, cervical cerclage)
Are the palms and soles usually involved in congenital candidiasis?
Yes
Are sucking blisters common or uncommon?
Common! They occur in one in every 250 newborns
Where do sucking blisters usually occur?
Radial forearm, wrist, hands, and fingers
Can fetal scalp electrodes cause scalp ulceration?
Yes; usually the erosions are superficial, but occasionally frank ulcers may ensue
When is the typical onset of acropustulosis of infancy?
Between 3 - 6 months
Are the skin lesions of acropustulosis of infancy pruritic?
Yes! They’re highly pruritic
Does acropustulosis of infancy occur more commonly in light-skinned or dark-skinned infants?
It affects darkly pigmented male infants most often
Does the history or recurrent crops of vesiculopustules fit with the diagnosis of acropustulosis of infancy?
Yes
What is the most important infectious disease that needs to be excluded in a patient with suspected acropustulosis of infancy?
Scabies
How can acropustulosis be treated? List a topical medication and two systemic medications.
Superpotent topical steroids (applied sparingly and intermittently), oral antihistamines, and dapsone (only if the eruption is severe because usually the risks outweight the benefits)
What dose of dapsone should be used in children with acropustulosis of infancy, if the risks of methemoglobinemia and hemolysis don’t outweigh the benefits of treatment?
1 - 2 mg/kg/day
What part of the body is most commonly involved in eosinophilic pustular folliculitis in infancy?
Scalp and face
Does eosinophilic pustular folliculitis in infancy resolve spontaneously?
Yes; the natural history is similar to acropustulosis of infancy, with spontaneous resolution within 3 - 5 years
Is there a relationship to HIV in infants with eosinophilic pustular folliculitis in infancy?
Yes, but the relationship is very rare (the relationship is much more frequent in adults)
True or false: congenital Langerhans cell histiocytosis is present at birth.
True
Is congenital Langerhans cell histiocytosis usually limited to the skin?
Yes
Other than congenital self-healing reticulohistiocytosis, name another synonymn (this one eponymous) for congenital Langerhans cell histiocytosis.
Hashimoto-Pritzker disease; both congenital self-healing reticulohistiocytosis and Hashimoto-Pritzker disease are recognized to be part of the Langerhans cell histiocytosis spectrum, thus they are now considered synonymns
Which two stains are most commonly used to identify Langerhans cells histologically?
CD1a and S100
What is the eponymn associated with incontinentia pigmenti?
Bloch-Sulzberger syndrome
How is Bloch-Sulzberger syndrome inherited?
X-linked dominant, thus it’s lethal in the vast majority of male fetuses (exceptions include Klinefelter babies with XXY phenotype and X mosaicism for the mutation)
What are the four cutaneous phases of incontinentia pigmenti?
Vesicular, verrucous, hyperpigmented, and hypopigmented
What gene is mutated in Bloch-Sulzberger syndrome?
NEMO (NF-kappa beta essential modulator)
What is the chromosomal locus for NEMO?
Xq28
What are the most common extracutaneous sites of involvement in patients affected by Bloch-Sulzberger syndrome?
Dental, ophthalmologic, neurologic, and musculoskeletal
*All patients hould have a baseline opthalmologic exam and well as periodic neurodevelopmental and dental evaluations
What is the eponymous name for hyperimmunoglobin E syndrome?
Job syndrome
List the four classic features of Job syndrome.
Cold abscesses, dermatitis, recurrent infections, and elevated serum IgE
*Note: these features are often not present during the first few months of life
What is the other name for oculo-oral-genital syndrome?
Behcet’s disease
Can Behcet’s disease occur in newborns?
Yes, usually in infants of mothers with known Behcet’s disease
Pyoderma gangrenosum occurs only rarely in infants. When it does occur, what is the most common location?
Perineum
What’s the prognosis for restrictive dermopathy that occurs in a newborn?
It’s fatal
What is the infectious etiology of noma neonatorum?
Pseudomonas aeruginosa
True or false: noma neonatorum usually produces necrotic ulcerations of the orofacial and anogenital regions.
True
Occlusion or spasm of which artery is thought to be responsible for perinatal gangrene of the buttock?
Internal iliac artery
What are the possible causes of vesiculopustular disease in the newborn? Hint: think broad categories
1) infectious (eg. bacterial, viral, fungal, parasitic), 2) non-infectious (eg. acropustulosis of infancy, eosinophilic pustular folliculitis in infancy, etc.), 3) transient (erythema toxicum neonatorum, miliaria crystallina, etc.)
What are the possible causes of bullae, erosions and ulcerations in the newborn? Hint: think broad categories
1) infection (eg. bacerial, viral), 2) transient (sucking blisters, perinatal trauma), 3) rare (epidermolysis bullosa, mastocytosis, etc.)
What % of full term neonates are affected by erythema toxicum neonatorum?
50%
What is the treatment for ETN?
None, resolves in a few days
What are the 3 phases of TNPM?
1) at delivery, small vesiculopustules on chin/forhead, neck, back, shins, 2) hyperpigmented macules with fine collarettes of scale, 3) residual hyperpigmented brown macules/PIH
What is the treatment for TNPM?
ne, benign, self-limiting
What are the 3 variants of miliaria?
1) miliaria crystallina, 2) miliaria rubra, 3) miliaria profunda
What conditions are associated with miliaria?
xcessive warming, overswaddling, fever, occlusive dressing
What is the treatment for miliaria?
None, resolves on own. Prevention with avoidance of overheating/overswaddling
What are the 2 clinical patterns of Candida albicans infection?
1) neonatal candidiasis, acquired at delivery or postnatally, 2) congenital candidiasis, acquired in utero
What is the treatment for neonatal candidiasis?
Topical anti-yeast medication (eg. imidazole cream). Also closely monitor infants who were premature or of low birthweights to determine whether parenteral antifungal is needed
What is the treatment for sucking blisters?
Supportive, clears in days-weeks
What is the timeline like for lesions in acropustulosis of infancy?
Onset 3-6mths of age, appear in crops x 1-2wks, recur in 3-4wks, spontaneous involution by 3yo
What is the treatment for acropustulosis of infancy?
Sparing application of topical corticosteroids (potent, superpotent), PO antihistamines, dapsone
What is the treatment for EPF?
Symptomatic, potent topical steroids, PO antihistamines, resolves over 3-5yrs
What is the treatment for congenital Langerhans cell histiocytosis?
Rule out systemic involvement. In those whose disease is limited to the skin, spontaneous resolution in wks-mths, close observation for relapses
What is the gene mutation in incontinentia pigmenti?
NEMO gene (NF-kappaB essential modulator) on Xq28, which confers protection against apoptosis
Do you always have high circulating IgE levels in hyper-IgE syndrome?
No, they are classicially associated with high circulating levels (>2000IU/mL) of IgE and eosinophilia, but this may not be evident during early infancy
What systemic effects are associated with intrauterine epidermal necrosis?
Brain infarcts/leukomalacia, cardiomegaly, renal tubular necrosis
What is the gene mutation in restrictive dermopathy?
LMNA and ZMPSTE24 (FACE-1) gene, which encodes lamin A and endoprotease
What are the 2 forms of noma neonatorum?
1) Pseudomonas aeruginosa septicemia, 2) severe combined immmunodeficiency in Native American infants
