Chapter 103 - Neonatal dermatology part II Flashcards by Beatrice Maxine Chan

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

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

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

radiologic evaluation

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High risk factors of occult dysraphism (5)

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

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Intermediate risk factors for occult spinal dysraphism (4)

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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Low risk for occult spinal dysraphism (5)

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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For high risk for occult spinal dysraphism, ___ is recommended

MRI

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For intermediate risk for occult spinal dysraphism, ___ is recommended

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

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

Dermoid cysts

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Treatment for dermoid cyst

Surgical excision

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Dermoid cysts are not always apparent at birth.

True or False

True

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

Nasal glioma

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Most common presentation of spina bifida

Myelomeningocele

Neural tissue of spinal cord is present

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

Meningocele

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

Encephalocele

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

Human tails

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Examples of pseudotails

Sacrococcygeal teratoma

Myelomeningocele

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

False, only true human tails

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Most common branchial cleft anonaly

Branchial cysts

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

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Definitive treatment for branchial cleft anomalies and thyroglossal duct cysts

Surgical excision

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

Thyroglossal duct cysts

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Present with persistent drainage from umbilicus or with an umbilical mass

Urachal and omphalomesenteric remnants

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Failure of regression of urachus or fistula between bladder and umbilicus

Urachal cyst

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Umbilical polyp or fistula between ileum and umbilicus

Omphalomesenteric duct

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

Umbilical granuloma

Treatment for umbilical granuloma

AgNO3 chemical cautery

Distort central facial features

Nasal glioma

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

Nasal dermoid cysts

Firm subQ mass at birth

Nasal glioma

Nasal glioma is not associated with intracranial connection. | True or False

True

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

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

Aplasia cutis congenita

Most common form of aplasia cutis congenita

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

Aplasia cutis when healed, lesions are usually atrophic. | True or False

True

Infants younger than ___ have immature immune systems

2 mos of age

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

50%; 75%

Vast majority of neonatal HSV are caused by

HSV Type 2

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

24 hours

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

1st

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

Omenn syndrome Severe combined immunodeficiency Hyperimmunoglobulinemia E syndrome DiGeorge syndrome

Treatment for SCID

Bone marrow transplantation before 3 to 4 months of age

Omenn syndrome is due to mutations in

RAG1 or RAG2 genes

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

Omenn syndrome

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

Hyperimmunoglobulinemia E syndrome

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

Seborrheic dermatitis

Seborrheic dermatitis remits spontaneously within ___ years of life

First 1-2

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

Diaper dermatitis

Treatment of diaper dermatitis

Barrier creams, frequent diaper changes

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

Candidal intertrigo

Treatment of Candidal intertrigo

Keep intertriginous areas dry | Topical antifungal agents

ABCs in treatment of diaper dermatitis

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

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

Cradle cap

Most common cause of diaper dermatitis

Irritant diaper dermatitis

Common contact allergens in diaper dermatitis (4)

Preservatives Fragrances Rubber additives Disperse dyes

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

3 days

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

Pseudoverrucous papules and nodules

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

Jacquet erosive dermatitis

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

Granuloma gluteale infantum

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

False, no true granulomas

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

Granuloma gluteale infantum

Infantile digital fibromas spares __(2)__

Thumbs and great toes

Stains for infantile digital fibroma

Masson trichome | Phosphotungstic acid-hematoxylin

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

A B C

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

Pseudo Darier sign (80%)

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

Neuroblastoma

Biopsy result of neuroblastoma

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

Immunohistochemical marker for neuroblastoma

Neuron specific enolase

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

60%

Sheets of atypical mononuclear cells infiltrating the dermis or subcutaneous fat

Congenital leukemia

Exceptionally rare form of mast cell disease in infants or children

Telangiectasia macularis eruptiva perstans

Brisk stroking of affected skin will result in mast cell drgranulation

Darier sign

Special stains of cutaneous mastocytosis

Toluidine blue Giemsa CKit (CD117)

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

100

More sensitive indicator of systemic mast cell disease in children

Enlargement of liver, spleen

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

Subcutaneous fat necrosis of newborn

Subcutaneous fat necrosis of newborn biopsy shows

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

Complication of subcutaneous fat necrosis of newborn

Hypercalcemia

Diffuse skin hardening in a sick premature newborn after 24 hours

Sclerema neonatorum

Sclerema neonatorum is common in patients with

Critically ill Premature neonates Sepsis Hypoglycemia Acidosis

Biopsy of sclerema neonatorum

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

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

Cutis marmorata telangiectatica congenita