Chapter 35 - Vesiculobullous and Erosive Diseases in the Newborn Flashcards Preview

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Flashcards in Chapter 35 - Vesiculobullous and Erosive Diseases in the Newborn Deck (83):
1

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

2

How old does a newborn have to be in order to catch scabies?

Usually 3-4 weeks or older

3

When does erythema toxicum neonatorum usually present?

Within 24-48 hours, but can be present from birth to two weeks

4

Which babies are affected by erythema toxicum neonatorum: term infants or preterm infants?

Term infants

5

Does erythema toxicum neonatorum affect the palms and soles?

No

6

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

7

What percentage of full-term neonates are affected by erythema toxicum neonatorum?

50%

8

Should erythema toxicum neonatorum be treated?

No; it resolves spontaneously without therapy within 1-2 days

9

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

10

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

11

Is transient neonatal pustular melanosis predominantly neutrophilic or eosinophilic?

Neutrophilic

12

Are the palms and soles affected in transient neonatal pustular melanosis?

Yes; there's widespread involvement, including the palms and soles

13

When does transient neonatal pustular melanosis typically develop?

Skin lesions are virtually always present at birth

14

Histologically, where does the pusutle occur in transient neonatal pustular melanosis?

Subcorneal region

15

Is treatment necessary for transient neonatal pustular melanosis?

No; it's a benign condition that resolves spontaneously

16

What are the three variants of miliaria?

Miliaria crystallina, miliaria rubra, and miliaria profunda

17

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

18

What is the other term for "malphigian layer"?

Spinous layer

19

What is the synonym for neonatal cephalic pustulosis?

Neonatal acne

20

When is the typical onset for neonatal cephalic pustulosis?

Within the first 2-3 weeks of life

21

Are comedones present in neonatal cephalic pustulosis?

No; it's a non-comedonal pustular eruption

22

How long does it usually take for neonatal cephalic pustulosis to resolve?

Weeks to months; therapy is not usually necessary

23

Which infectious organisms have been implicated in neonatal cephalic pustulosis?

M. furfur, M. sympodialis, and M. globosa

24

Normally no treatment is necessary for neonatal cephalic pusulosis, however, if treatment is desired, what topical medication can help?

A topical imidazole

25

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"

26

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

27

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

28

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)

29

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)

30

Are the palms and soles usually involved in congenital candidiasis?

Yes

31

Are sucking blisters common or uncommon?

Common! They occur in one in every 250 newborns

32

Where do sucking blisters usually occur?

Radial forearm, wrist, hands, and fingers

33

Can fetal scalp electrodes cause scalp ulceration?

Yes; usually the erosions are superficial, but occasionally frank ulcers may ensue

34

When is the typical onset of acropustulosis of infancy?

Between 3 - 6 months

35

Are the skin lesions of acropustulosis of infancy pruritic?

Yes! They're highly pruritic

36

Does acropustulosis of infancy occur more commonly in light-skinned or dark-skinned infants?

It affects darkly pigmented male infants most often

37

Does the history or recurrent crops of vesiculopustules fit with the diagnosis of acropustulosis of infancy?

Yes

38

What is the most important infectious disease that needs to be excluded in a patient with suspected acropustulosis of infancy?

Scabies

39

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)

40

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

41

What part of the body is most commonly involved in eosinophilic pustular folliculitis in infancy?

Scalp and face

42

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

43

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)

44

True or false: congenital Langerhans cell histiocytosis is present at birth.

True

45

Is congenital Langerhans cell histiocytosis usually limited to the skin?

Yes

46

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

47

Which two stains are most commonly used to identify Langerhans cells histologically?

CD1a and S100

48

What is the eponymn associated with incontinentia pigmenti?

Bloch-Sulzberger syndrome

49

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)

50

What are the four cutaneous phases of incontinentia pigmenti?

Vesicular, verrucous, hyperpigmented, and hypopigmented

51

What gene is mutated in Bloch-Sulzberger syndrome?

NEMO (NF-kappa beta essential modulator)

52

What is the chromosomal locus for NEMO?

Xq28

53

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

54

What is the eponymous name for hyperimmunoglobin E syndrome?

Job syndrome

55

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

56

What is the other name for oculo-oral-genital syndrome?

Behcet's disease

57

Can Behcet's disease occur in newborns?

Yes, usually in infants of mothers with known Behcet's disease

58

Pyoderma gangrenosum occurs only rarely in infants. When it does occur, what is the most common location?

Perineum

59

What's the prognosis for restrictive dermopathy that occurs in a newborn?

It's fatal

60

What is the infectious etiology of noma neonatorum?

Pseudomonas aeruginosa

61

True or false: noma neonatorum usually produces necrotic ulcerations of the orofacial and anogenital regions.

True

62

Occlusion or spasm of which artery is thought to be responsible for perinatal gangrene of the buttock?

Internal iliac artery

63

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.)

64

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.)

65

What % of full term neonates are affected by erythema toxicum neonatorum?

50%

66

What is the treatment for ETN?

None, resolves in a few days

67

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

68

What is the treatment for TNPM?

ne, benign, self-limiting

69

What are the 3 variants of miliaria?


1) miliaria crystallina, 2) miliaria rubra, 3) miliaria profunda

70

What conditions are associated with miliaria?

xcessive warming, overswaddling, fever, occlusive dressing

71

What is the treatment for miliaria?

None, resolves on own. Prevention with avoidance of overheating/overswaddling

72

What are the 2 clinical patterns of Candida albicans infection?

1) neonatal candidiasis, acquired at delivery or postnatally, 2) congenital candidiasis, acquired in utero

73

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

74

What is the treatment for sucking blisters?

Supportive, clears in days-weeks

75

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

76

What is the treatment for acropustulosis of infancy?

Sparing application of topical corticosteroids (potent, superpotent), PO antihistamines, dapsone

77

What is the treatment for EPF?

Symptomatic, potent topical steroids, PO antihistamines, resolves over 3-5yrs

78

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

79

What is the gene mutation in incontinentia pigmenti?

NEMO gene (NF-kappaB essential modulator) on Xq28, which confers protection against apoptosis

80

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

81

What systemic effects are associated with intrauterine epidermal necrosis?

Brain infarcts/leukomalacia, cardiomegaly, renal tubular necrosis

82

What is the gene mutation in restrictive dermopathy?

LMNA and ZMPSTE24 (FACE-1) gene, which encodes lamin A and endoprotease

83

What are the 2 forms of noma neonatorum?

1) Pseudomonas aeruginosa septicemia, 2) severe combined immmunodeficiency in Native American infants