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7th > Chapter 103 - Neonatal dermatology part II > Flashcards

Chapter 103 - Neonatal dermatology part II Flashcards

1
Q

Single minor congenital anomalies occur in approximately ___% of all newborns

A

15

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2
Q

___ or more minor congenital anomalies is unusual and warrants a complete thorough physical examination to rule out other congenital abnormalities

A

3

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3
Q

For midline nasal and facial lesions, ___ is indicated to rule out intracranial connection before skin biopsy is performed

A

radiologic evaluation

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4
Q

High risk factors of occult dysraphism (5)

A 
2 or more lesions of any kind
One lesion + spinal cord dysfunction
Lipoma
Tail
Dermal sinus
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5
Q

Intermediate risk factors for occult spinal dysraphism (4)

A

Atypical sacral dimple (>/=5mm diameter in location, >/=2.5 cm away from anus)
Aplasia cutis congenita
Overting hamartoma
Deviated gluteal cleft

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6
Q

Low risk for occult spinal dysraphism (5)

A 
Port wine stain
Hypertrichosis
Pigmented nevus
Simple sacral dimple (<5mm diamter, 2.5 cm or closer to the anus)
Mongolian spot
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7
Q

For high risk for occult spinal dysraphism, ___ is recommended

A

MRI

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8
Q

For intermediate risk for occult spinal dysraphism, ___ is recommended

A

Ultrasonography if younger than 4 mos old then MRI if abnormal
MRI if older than 4mos

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9
Q

Congenital anonalies that arise along planes of embryonic fusion of the face and scalp

A

Dermoid cysts

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10
Q

Treatment for dermoid cyst

A

Surgical excision

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11
Q

Dermoid cysts are not always apparent at birth.

True or False

A

True

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12
Q

Firm, skin-colored to erythematous nodules on the nose composed of heterotopic neural tissue

A

Nasal glioma

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13
Q

Most common presentation of spina bifida

A

Myelomeningocele

Neural tissue of spinal cord is present

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14
Q

Midline, formed when the meninges and CSF herniate through a defect in the calvarium or vertebrae

A

Meningocele

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15
Q

Herniation of neural tissue along with meninges and CSF through a calvarial defect

A

Encephalocele

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16
Q

Vestigial appendages that may be composed of adipose tissue, blood vessels, muscle fibers, and nerves

A

Human tails

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17
Q

Examples of pseudotails

A

Sacrococcygeal teratoma

Myelomeningocele

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18
Q

Both true human tails and paeudotails are associated with spinal dysraphism and warrants MRI examination
True or False

A

False, only true human tails

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19
Q

Most common branchial cleft anonaly

A

Branchial cysts

2nd branchial arch - sternocleidomastoid on lateral aspect of the neck

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20
Q

Definitive treatment for branchial cleft anomalies and thyroglossal duct cysts

A

Surgical excision

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21
Q

Congenital neck anonalies that present as midline mass arising anywhere fron suprasternal notch to posterior tongue

A

Thyroglossal duct cysts

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22
Q

Present with persistent drainage from umbilicus or with an umbilical mass

A

Urachal and omphalomesenteric remnants

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23
Q

Failure of regression of urachus or fistula between bladder and umbilicus

A

Urachal cyst

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24
Q

Umbilical polyp or fistula between ileum and umbilicus

A

Omphalomesenteric duct

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25
Q

Common abnormalities of umbilicus that presents with persistent friable granulation tissue involving umbilicus after separation of umbilical cord

A

Umbilical granuloma

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26
Q

Treatment for umbilical granuloma

A

AgNO3 chemical cautery

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27
Q

Distort central facial features

A

Nasal glioma

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28
Q

Associated with dermal pit or sinus with significant risk of intracranial extension

A

Nasal dermoid cysts

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29
Q

Firm subQ mass at birth

A

Nasal glioma

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30
Q

Nasal glioma is not associated with intracranial connection.

True or False

A

True

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31
Q

Ring of darker and/or coarser terminal hairs on the scalp typically surrounding ACC, dermoid cyst, encephalocele, meningocele, or heterotopic brain tissue

A

Hair collar sign

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32
Q

Localized areas of absence of the skin and sometimes the underlying subQ tissues and bones

A

Aplasia cutis congenita

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33
Q

Most common form of aplasia cutis congenita

A

Membranous aplasia cutis
(Oval, sharply, marginated atrophic macules seen on midline of posterior scalp; may be ulcerated or crusted with thin shiny membranous covering)

34
Q

Aplasia cutis when healed, lesions are usually atrophic.

True or False

35
Q

Infants younger than ___ have immature immune systems

A

2 mos of age

36
Q

Untreated neonatal herpes simplex infection has a ___% mortality rate with __% having neurologic sequelae

37
Q

Vast majority of neonatal HSV are caused by

A

HSV Type 2

38
Q

Vesicles that appear during the first __ hours suggest in utero acquistion of HSV

39
Q

Atopic dermatitis onset is usually delayed until after __ months of life

40
Q

Several primary immunodeficiency syndromes are associated with neonatal erythroderma (4)

A

Omenn syndrome
Severe combined immunodeficiency
Hyperimmunoglobulinemia E syndrome
DiGeorge syndrome

41
Q

Treatment for SCID

A

Bone marrow transplantation before 3 to 4 months of age

42
Q

Omenn syndrome is due to mutations in

A

RAG1 or RAG2 genes

43
Q

Characterized by failure to thrive, chronic diarrhea, lymphadenopathy, hepatosplenomegaly

A

Omenn syndrome

44
Q

Diffuse eczematous dermatitis in infancy, atopy, mucocutaneous candidiasis, and recurrent staphylococcal infections

A

Hyperimmunoglobulinemia E syndrome

45
Q

Common and benign dermatoses, as early as first few weeks of life often on diaper and intertriginous areas

A

Seborrheic dermatitis

46
Q

Seborrheic dermatitis remits spontaneously within ___ years of life

47
Q

Occurs at any areas involving convexities of genitalia with sparing of folds

A

Diaper dermatitis

48
Q

Treatment of diaper dermatitis

A

Barrier creams, frequent diaper changes

49
Q

Bright red erythematous moist papules and plaques with satellitosis affecting both convexities and skin folds

A

Candidal intertrigo

50
Q

Treatment of Candidal intertrigo

A

Keep intertriginous areas dry

Topical antifungal agents

51
Q

ABCs in treatment of diaper dermatitis

A 
A-ir
B-arrier cream
C- leansing
D-iaper
E-ducation
52
Q

Most common presentation is development of greasy yellow scale with mild underlying erythema on scalp

A

Cradle cap

53
Q

Most common cause of diaper dermatitis

A

Irritant diaper dermatitis

54
Q

Common contact allergens in diaper dermatitis (4)

A

Preservatives
Fragrances
Rubber additives
Disperse dyes

55
Q

Candida typically exacerbate any type of diaper dermatitis present for longer than __

56
Q

Flat topped, skin colored papules that develop in diaper and perianal area in patients whose skin is chronically exposed to moisture, in children with prolonged urinary or fecal incontinence

A

Pseudoverrucous papules and nodules

57
Q

Uncommon severe dermatitis that is characterized by well-demarcated , punched out ulcers and erosions

A

Jacquet erosive dermatitis

58
Q

Uncommon diaper rash characterized by reddish purplish nodules of different sizes (0.5-3cm) on convexities of diaper area in 2 to 9 month old infants

A

Granuloma gluteale infantum

59
Q

Biopsy of granuloma gluteale infantuma shows true granulomas.
True or False

A

False, no true granulomas

60
Q

Unusual reaction to irritant factors, Candida infection, topical corticosteroid use

A

Granuloma gluteale infantum

61
Q

Infantile digital fibromas spares __(2)__

A

Thumbs and great toes

62
Q

Stains for infantile digital fibroma

A

Masson trichome

Phosphotungstic acid-hematoxylin

63
Q

Matching type

  1. Resolves spontaneously
  2. Low recurrence rate
  3. High recurrence rate

A. Infantile Myofibromatosis
B. Fibrous Hamartoma of Infancy
C. Infantile Digital Fibroma

64
Q

Brisk rubbing of congenital smooth muscle tumor results in a transient piloerection and induration

A

Pseudo Darier sign (80%)

65
Q

Second most common solid tumor of childhood, 25% are congenital

A

Neuroblastoma

66
Q

Biopsy result of neuroblastoma

A

Small round cells with larger atypical nuclei; if malignant, may form pseudorosette

67
Q

Immunohistochemical marker for neuroblastoma

A

Neuron specific enolase

68
Q

Multiple blue to purple skin nodules are found in __% of newborn with congenital leukemia

69
Q

Sheets of atypical mononuclear cells infiltrating the dermis or subcutaneous fat

A

Congenital leukemia

70
Q

Exceptionally rare form of mast cell disease in infants or children

A

Telangiectasia macularis eruptiva perstans

71
Q

Brisk stroking of affected skin will result in mast cell drgranulation

A

Darier sign

72
Q

Special stains of cutaneous mastocytosis

A

Toluidine blue
Giemsa
CKit (CD117)

73
Q

Bone marrow biopsy is indicated for serum tryptase levels greater than ___ ng/ml

74
Q

More sensitive indicator of systemic mast cell disease in children

A

Enlargement of liver, spleen

75
Q

Firm, red to purple subcutaneous nodules or plaques on back, cheeks, buttocks, arms, thighs within the first 2 weeks of life and resolves spontaneously over several weeks

A

Subcutaneous fat necrosis of newborn

76
Q

Subcutaneous fat necrosis of newborn biopsy shows

A

Lobular fat necrosis with needle shaped clefts in lipocytes and mixed inflammation with lymphocytes, macrophages, and giant cells

77
Q

Complication of subcutaneous fat necrosis of newborn

A

Hypercalcemia

78
Q

Diffuse skin hardening in a sick premature newborn after 24 hours

A

Sclerema neonatorum

79
Q

Sclerema neonatorum is common in patients with

A

Critically ill Premature neonates
Sepsis
Hypoglycemia
Acidosis

80
Q

Biopsy of sclerema neonatorum

A

Needle-like crystals within lipocytes but with no associated inflammatory infiltrate or fat necrosis

81
Q

Characterized by persistent reticulated atrophic violaceous vascular patches with telangiectasias and ulceration on lower extremities improves on first 2 years

A

Cutis marmorata telangiectatica congenita

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