Chapter 103 - Neonatal dermatology part II Flashcards
Single minor congenital anomalies occur in approximately ___% of all newborns
15
___ or more minor congenital anomalies is unusual and warrants a complete thorough physical examination to rule out other congenital abnormalities
3
For midline nasal and facial lesions, ___ is indicated to rule out intracranial connection before skin biopsy is performed
radiologic evaluation
High risk factors of occult dysraphism (5)
2 or more lesions of any kind One lesion + spinal cord dysfunction Lipoma Tail Dermal sinus
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
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
For high risk for occult spinal dysraphism, ___ is recommended
MRI
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
Congenital anonalies that arise along planes of embryonic fusion of the face and scalp
Dermoid cysts
Treatment for dermoid cyst
Surgical excision
Dermoid cysts are not always apparent at birth.
True or False
True
Firm, skin-colored to erythematous nodules on the nose composed of heterotopic neural tissue
Nasal glioma
Most common presentation of spina bifida
Myelomeningocele
Neural tissue of spinal cord is present
Midline, formed when the meninges and CSF herniate through a defect in the calvarium or vertebrae
Meningocele
Herniation of neural tissue along with meninges and CSF through a calvarial defect
Encephalocele
Vestigial appendages that may be composed of adipose tissue, blood vessels, muscle fibers, and nerves
Human tails
Examples of pseudotails
Sacrococcygeal teratoma
Myelomeningocele
Both true human tails and paeudotails are associated with spinal dysraphism and warrants MRI examination
True or False
False, only true human tails
Most common branchial cleft anonaly
Branchial cysts
2nd branchial arch - sternocleidomastoid on lateral aspect of the neck
Definitive treatment for branchial cleft anomalies and thyroglossal duct cysts
Surgical excision
Congenital neck anonalies that present as midline mass arising anywhere fron suprasternal notch to posterior tongue
Thyroglossal duct cysts
Present with persistent drainage from umbilicus or with an umbilical mass
Urachal and omphalomesenteric remnants
Failure of regression of urachus or fistula between bladder and umbilicus
Urachal cyst
Umbilical polyp or fistula between ileum and umbilicus
Omphalomesenteric duct
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
- Resolves spontaneously
- Low recurrence rate
- 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
