Skin Devo Flashcards

1
Q

Precursors for Skin

A

Head - Neural crest & Head mesoderm
Trunks & Limbs - Somatic
Back - Dermatome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is skin at 3rd week of development?

A

Single layer of surface ectoderm

Permeable layer that allows fluid to pass through

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Periderm & Intermediate layer Formation

A

At wks 4-8 thin layer of peridermal cells begin to cover surface ectoderm
Peridermal cells develop micorvilli to absorb nutrients from fluid
At wks 11-12: Mitosis from basal to form intermediate layer, skin starts become barrier against amnionic fluid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Definitive layer formation

A

All the definitive layers form during 4-6 months (Fetal period)
Peridermis goes away, replaced by keratinocyte
Vernix Caseosa - White, sticky covering around fetus, sebaceous secretion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What cells migrate as dermis matures?

A

Langerhan’s Cells - End of fetal period from bone marrow
Melanocyte
Merkel cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Piebaldism (Genetics, Mechanism, Findings)

A

Autosomal dominant, mutation of the KIT proto-oncogene
Results in impaired migration of melanocytes
Results in depigmented congenital patches often with speckles within, stable, nonprogressive, white forelock of hair is typical
History is what distinguishes with vitiligo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Waardenburg syndrome (Genetics, Mechanisms, Findings)

A

Rare autosomal dominant, multiple genes implicated
Abnormal development of melanocyte
Results primarily with achromia (White patches of skin/hair)
Also has deafness, heterochromia irides, dystopia canthorum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Developmental history of dermis

A

Fibroblasts migrate in
Begin to produce collagen
Form papillary and reticular (thicker fibers) regions in dermis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Port-wine stain (Mechanism, Incidence)

A

Malformed, ectatic, dilated capillary to venule sized blood vessels
Always present at birth
Occur in about 0.3%-0.6%
Tend to darken and thicken over time

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Facial Port-wine stain distribution

A

V1 - Ophthalmic branch: Can extend to conjunctiva
V2 - Maxillary branch: Less risk for complications (watershed risk with lower eyelid)
V3 - Mandibular branch - May extend to oral mucosa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Sturge-Weber Syndrome (Facial Association, Neurologic findings, Occular findings)

A

V1 dermatome associated
Neurological: Seizures, developmental delay, migraine headaches, tram track calcifications (calcification of the occipital and/or temporal cortex)
Ocular: Congenital glaucoma, increased choroidal vascularity (tomato ketchup spot)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Infantile Hemangioma (Incidence, Appearance, Complication)

A

Incidence - Most common vascular tumor: 4-5% estimated incidence
Appearance - Endothelial-like proliferating cells that become clinically visible within the first months of life
Complications - Failure to open eye in early months can lead to permanent blindness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Infantile Hemaginoma Growth Cycle

A
Birth - Appears as a white flat patch with fine, pink-red vessels
Pallor - Due to local vasoconstriction
1-2 wks: Proliferation begins
Growth phase variable: 3-9 months
Slow involution (not resolution)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

PHACE Syndrome

A
Posterior fossa abnormalities
Hemangioma (segmental)
Arterial Anomalies
Cardiac Anomalies
Eye Anomalies
Sternal clefting/supraumbilical raphe
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Formation of Hair Follicles

A
Thickening of epidermis to form hair bud
Extends into the mesenchyme to form hair bulb
Mesenchyme joins to form dermal papilla
Hair forms out
Erector pillae forms from ectoderm
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Formation of Sweat gland

A

Budding from basal layer
Solid cylindrical downgrowth
Form a lumen and a secretory coil

Eccrine - Enters into the surface
Apocrine - Enters into the hair folicle

17
Q

Eccrine vs Apocrine (Mechanism of secretion and Location)

A

Eccrine - Merocrine (Cross-membrane) secretion; palms, soles

Apocrine - Apocrine (partial cytoplasm is removed) secretion; axilla, genitalia

18
Q

Formation of nails

A

Nail fold surrounds nail field

Nail plate comes underneath and forms eponychium

19
Q

Ectodermal Dysplasia - Number of types, what structures can be affected

A

Over 150 rare syndromes altering in 2 or more structures from embryonic ectoderm
Defects in hair, teeth, nails, sweat glands, lens of the eye

20
Q

Hypohidrotic Ectodermal Dysplasia (Genetics, incidence, pathway)

A

X-Linked Recessive
Most common form of ED encountered by clinicians
Ectodysplasin Signaling Pathway: Ectodysplasin-A1 (EDA-A1), Eda-A1 receptor (EDAR), EDAR associated death domain

21
Q

HED Findings

A
Square forehead with frontal bossing
Flattened nasal bridge
Low-lying ears
Skin is thin and dry
Sparse hair
Hypo-anodontia/Peg Teeth
Decreased ability to sweat
22
Q

HED Management

A

Avoid overheating
Consult dentistry
Recommend families to contact National Foundation for Ectodermal Dysplasias