Pathophys-Day 5 Ages I Flashcards
Ectoderm divides into __ and __
Extoderm and neuroectoderm (neural crest and neural tube)
Epidermis is formed from ?
ectoderm
5 layers of newly-formed epidermis
Strata basale, spinulosum, granulosum, lucidum, corneum
Lucidum is only on hands and disappears other places
Explain how epidermis changes during embryogenesis and later
Wk 6 - bilayered w periderm and basal layer
wk 8 - stratification begins - intermediate and basal layers
3rd trimester: terminally diff, filaggrin expressed
Week 8 defects during embryonic epidermal genesis lead to
ectodermal dysplasia
Ectodermal dysplasia presentation?
Hair, teeth, bone, skin deformities:
Clefting
Ectrodactyly
Sparse hair
Defects in epidermal genesis in the third trimester cause:
Ichthyoses
Ichthyoses presentation
Hyperlinear palms and soles; inherited, due to filaggrin mutation
Melanocytes originate in the _
neural crest
Where all do melanocytes migrate to?
Ear
Eye
Skin
Meninges
Describe the three categories of abnormal melanocyte development
Origin/migration/survival: patches of no pig
Melanin production: albinism
Melanosome formation/move: pigment dilution
What is piebaldism?
Patches of depigmentation (splotchy albinism!) from melanocyte mutation
Waardenberg syndrome?
Defective survival of melanocytes leads to patches of depigmentation
contrast to piebaldism>melano mutation
Presentation of Hermansky-Pudlak and Chediak-Higashi?
Silver hair from melanosome issues (remember - lysosome storage issues)
What are lines of Blaschko/Blaschkoid vesicles and when are they seen?
Migratory lines of melanocytes where mosaics have developed lighter skin
X-linked (lyonization)
Incontinentia pigmenti
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)