case studies

Question 1

What is the Luxol-fast blue stain used for?

Answer 1

It stains normal myelin blue

Question 2

What does it mean when there is no cellular infiltrate in an MS plaque?

Answer 2

It is a chrnic plaque and demyelination occured a long time ago

Question 3

what is a sevier-munger stain used for?

Answer 3

it highlights axonal processes, which are visible as black threadline structues
- axonal preservation is present in some plaques despite loss of myelin

Question 4

what is an important inflmmatiory mediator in the plaques of MS?

Answer 4

macrophages because the phagocytize lipid rich myelin debris

Question 5

what disease causes symmetrical myelin injury and spating of subcortical U fibers?

Answer 5

leukodystrophy

Question 6

metachromic leukodystrophy

Answer 6

arylsulfatase A deficiency

Question 7

Krabbe disease

Answer 7

glactocerebroside b-galactosidase deficiency

Question 8

arenoleukodytrophy

Answer 8

defect in peroxisomal membrane associted protein

Question 9

what is Wallerian degeneration?

Answer 9

a type of axonal degeneration in which axons degenerate in response to more proximal nerve injury (transection injury)

Question 10

what is primary axonal degeneration?

Answer 10

type of axonal degeneration that begins in the most distal portion of the axon and progresses in a proximal fashon (loss of sensation in a stocking glove pattern)

Question 11

myelin ovoids

Answer 11

degenerating axons and fragments of myelin characteristic feature of axonal peripheral neuropathies

Question 12

what place is common for a schwannoma?

Answer 12

CN VII AKA acoustic neuroma

Question 13

what are verocay bodies?

Answer 13

the neopastic nuclei of a schwanoma line up in irregular palisades

Question 14

what neoplasm are associted wuth Antoni A and Antoni B

Answer 14

schwanomas

Question 15

what cells are in the stratum basle?

Answer 15

keratinocytes, melanocytes, and merkel cell

Question 16

what is in the stratum germinativum?

Answer 16

germ/stem cells to make the keratinocytes

Question 17

what is in the stratum spinosum?

Answer 17

new keratinocytes and desmosomes

Question 18

what is is the stratum granulosum?

Answer 18

granular layer with protein rich keratohylanie granules

Question 19

what is in the stratum lucidem?

Answer 19

keratinocyes

Question 20

what is in the stratum corneum?

Answer 20

anucleated cells with keratin

Question 21

what is a keratinocye?

Answer 21

specialized epithelias cell that has both basal cells and squamous cells `

Question 22

where are merkel cells present?

Answer 22

specialized regions: lips, oral cavity, palmar skin

associated with terminal neuronal axon

Question 23

where are the greatest number of ecccrine glands?

Answer 23

chest
face
back

Question 24

what is the job of eccrine glands?

Answer 24

regulate body temp

Question 25

where are appocrine sweat glands?

Answer 25

axillae, face, abdomen, genital, scalp

Question 26

what is in the papillary layer of the dermis?

Answer 26

loose conective tissue right beneath the epidermis

Question 27

what is in the reticular later of the dermis?

Answer 27

dense dermal collagen/ much thicker and fibrotic

Question 28

what is a becker nevus and what should you not confuse it with?

Answer 28

it is a solitary lesion that beaks up into smaller macules at the periphery dont confuse with cafe au lait

Question 29

what is the tyndall effect?

Answer 29

blue color due to scattering of shorter wavelengths by dermal melanin

Question 30

what is a nevus of Ota vs Ito

Answer 30

ota- trigeminal nerve 1st and 2nd divisions | Ito- posterior supraclavicular and lateral brachiocutaneous nerves

Question 31

what is a benign nevus?

Answer 31

a nevus that does not mature, it stays at the dermal epidermal junction

Question 32

what 2 mutations are associated with dysplastic nevi syndrome?

Answer 32

P16 (CDKN2A) or CDK4 CDK4- resistant to being turned off by P16 P16- can't block proliferation - both cause uncontrolled proliferation

Question 33

what is the inheritance pattern in dysplastic nevi syndrome?

Answer 33

anutosomal dominant

Question 34

what is prognosis for malignant melonoma 100% related to?

Answer 34

vertical growth/breslows thickening and TNM

Question 35

lentigo maligna

Answer 35

indolent, usually on the face, seen in elderly | does not spread rapidly

Question 36

superficial spreading

Answer 36

most commom | seen in sun exposed areas

Question 37

acral/mucosal lentiginouse

Answer 37

unrelated to sun exposure, in anogenital regions

Question 38

what is considered a good prognosis in breslow depth?

Answer 38

< 1.7 mm

Question 39

what is clark's levels?

Answer 39

10 yr survival based on border

Question 40

what gene mutations predispose an individual to malignant melonoma?

Answer 40

CDKN2A (P16) allows for no turning off of proliferation mutated in 40% of familial melonoma aberrant increaes in RAS and P13/AKT signanling

Question 41

what 3 tummor suppressors does P16 code for? why is that importaint?

Answer 41

P15/INK4b P16/INK4a- normally enhances activity og TSGs of Rb family inhibiting CDK4 P14/ARF- enhances activity of p53 by inhibiting activity og MDM2 * all play a role in suppressing proloferation, if mutated cells can grow with no control

Question 42

what are the gene mutations associated with fair skinned individuals?

Answer 42

MCIR- melanocortin 1 receptor ASLP-regulation of melanocortin rc signaling TYR-tyrosine enzyme mutation

Question 43

dermatosis papulosa nigra

Answer 43

a type of seborrheic keratosis, but with multiple small lesions on the face

Question 44

what gene mutation is assocted with seborrheic keratosis?

Answer 44

FGFR3 gene- fibroblast growth factor rc gene

Question 45

what is paraneoplastic syndrome?

Answer 45

when seborrheic keratosis occurd explosivly in large numbers results from a growing tumor it is called the leser-Trelat sign and is due to stimulation of transforming growth factor alpha from GI carcinoma

Question 46

what are the genes associated with bening appendage tumors? why imp?

Answer 46

TSG and PTEN because it can predispose ot internal malignancy

Question 47

what us Muir Torre syndrome and what is is associted with?

Answer 47

a subset of heredity nonpolyposis carcinoma syndrome associated with sabaceous adenomas that can convert into adenomas

Question 48

actinic chelitis

Answer 48

keratosis of the lip

Question 49

impuimod

Answer 49

medication to stimulate the immune system byt stimulating toll like rcs before it turns into SCC -used in actinic keratosis

Question 50

what is bowen's disease?

Answer 50

SCC in situ- no invasion on the dermatoepidermal junction shaply defined red scaly plaques involves all layers of the epidermis

Question 51

what strain of HPV are associated with SCC?

Answer 51

5 and 8

Question 52

what are the 2 patterns of BCC?

Answer 52

1. multifocal growths-originate in epidermis, extend several square cm 2. nodular lesions-grow down into dermis, originate in follicular epithelium

Question 53

what is nevoid BCC syndrome and what gene muattions are associted?

Answer 53

gorlin syndrome, autosomal dominant chr9 PTCH mutations

Question 54

what does PTCH encode for?

Answer 54

a rc for sonic hedgehog gene, absence of PTCH causes activation of SMO, which leads to BCC

Question 55

what other conditions is NBCC associted with?

Answer 55

medulloblastomas and ovarian fibromas

Question 56

What is Non NBCC?

Answer 56

still has a genertic component 30% have PTCH mutations 40-60% have p53 mutations xeroderma pigmentosa has both PTCH and p53 mutations- doblue wammy for developing lesions ( constant proliferation and increased survival)