Pathophys-Day 5 Ages I

Question 1

Ectoderm divides into __ and __

Answer 1

Extoderm and neuroectoderm (neural crest and neural tube)

Question 2

Epidermis is formed from ?

Answer 2

ectoderm

Question 3

5 layers of newly-formed epidermis

Answer 3

Strata basale, spinulosum, granulosum, lucidum, corneum

Lucidum is only on hands and disappears other places

Question 4

Explain how epidermis changes during embryogenesis and later

Answer 4

Wk 6 - bilayered w periderm and basal layer

wk 8 - stratification begins - intermediate and basal layers

3rd trimester: terminally diff, filaggrin expressed

Question 5

Week 8 defects during embryonic epidermal genesis lead to

Answer 5

ectodermal dysplasia

Question 6

Ectodermal dysplasia presentation?

Answer 6

Hair, teeth, bone, skin deformities:
Clefting
Ectrodactyly
Sparse hair

Question 7

Defects in epidermal genesis in the third trimester cause:

Answer 7

Ichthyoses

Question 8

Ichthyoses presentation

Answer 8

Hyperlinear palms and soles; inherited, due to filaggrin mutation

Question 9

Melanocytes originate in the _

Answer 9

neural crest

Question 10

Where all do melanocytes migrate to?

Answer 10

Ear
Eye
Skin
Meninges

Question 11

Describe the three categories of abnormal melanocyte development

Answer 11

Origin/migration/survival: patches of no pig
Melanin production: albinism
Melanosome formation/move: pigment dilution

Question 12

What is piebaldism?

Answer 12

Patches of depigmentation (splotchy albinism!) from melanocyte mutation

Question 13

Waardenberg syndrome?

Answer 13

Defective survival of melanocytes leads to patches of depigmentation

contrast to piebaldism>melano mutation

Question 14

Presentation of Hermansky-Pudlak and Chediak-Higashi?

Answer 14

Silver hair from melanosome issues (remember - lysosome storage issues)

Question 15

What are lines of Blaschko/Blaschkoid vesicles and when are they seen?

Answer 15

Migratory lines of melanocytes where mosaics have developed lighter skin

X-linked (lyonization)

Question 16

Incontinentia pigmenti

Answer 16

XLD

Infants with IP are born with streaky, blistering areas. When the areas heal, they turn into rough bumps. Eventually, these bumps go away, but leave behind darkened skin, called hyperpigmentation. After several years, the skin returns to normal. In some adults, there may be areas of lighter colored skin (hypopigmentation).

Ocular, dental, CNS defects

Fatal in utero in males (only 1 X)

Question 17

Where does dermis develop from

Answer 17

ectoderm and mesoderm

Question 18

when is the dermal-epidermal junction fully functional?

Answer 18

12 wks

Question 19

WHen is the skin’s barrier function fully developed?

Answer 19

3 wks post-partum

Question 20

Important considerations to keep in mind for neonates and different dermal needs

Answer 20

1. SA ratio much higher - fast topical absorb
2. inc percutaneous absorp
3. Increased transepi water loss

Question 21

Considerations in the premature kid

Answer 21

Thinner stratum corneum
Longer than 3 wks for barrier fxn
Inc TEWL even comp to full-term
Inc infxn risk

Question 22

What is vernix caseosa?

Answer 22

Protective membrane used in utero to keep baby’s skin dry made of epithelium, sebaceous secretions, and shed lanugo hair

Question 23

What is cutis marmorata?

Answer 23

Reticulated skin appearance when baby is cold; goes away

Question 24

What is lanugo?

Answer 24

hairy baby

Question 25

Milia?

Answer 25

Little cysts in mouth, goes away

Question 26

What is mongolian spot?

Answer 26

Benign flat, congenital grey birthmark with wavy borders and irregular shape

Question 27

What is erythema toxicum neonatorum?

Answer 27

Benign, up to 50% babies

Question 28

What are miliaria?

Answer 28

Occlusion of eccrine glands when baby is wrapped to tightly and too warm

Question 29

What is seborrheic dermatitis?

Answer 29

Cradle cap

Question 30

How can you tell if dermatitis diaperis is from candida?

Answer 30

Satellite lesions with redness

Question 31

Gianotti-Crosti

Answer 31

rash in response to many different viruses, often EBV

Question 32

HSP?

Answer 32

Strep in kids, vasculitis, glomerular nephritis is possible

Question 33

Kawasaki disease

Answer 33

prob infectious Big thing: puffy hands and feet, desquamation, strawberry tongue, potential coronary anyeurism

Question 34

Atopic dermatitis pathogenesis

Answer 34

Type I HS Filaggrin mutations assoc with AD Barrier dysfunction leads to allergen exposure, immune dysregulation, and environmental interactions

Question 35

Atopic dermatitis presentation

Answer 35

=eczema Pruritis in past 12 months + 3 of: 1. generally dry skin 2. allergic rhinitis or asthma 3. onset before 2 4. hx of skin crease 5. visible flexural dermatitis

Question 36

Describe the infantile phase of AD

Answer 36

Cheeks, forehead, scalp, extensors NOT DIAPER AREA Itchy, ozzy, red

Question 37

Childhood phase of AD

Answer 37

FLEXOR surfaces, wrist, ankle, neck | Lichenification

Question 38

What are some features associated with childhood AD?

Answer 38

Denny-Morgan pleats: transverse eye lines Allergic shiners: rubbing eyes Allergic salute: rub nose w arm - crease Hyperlinear palms

Question 39

What are some complications of AD?

Answer 39

S aureus! HSV Molluscum contagiosum (poxvirus) ADHD

Question 40

Management of AD

Answer 40

``` Moisturization Bathing followed by immediate hydration and covering Avoid heat Calcineurin inhibitors Topical steroids ```