Skin Path CIS

Question 1

Biopsy of 3 of 100 tan to brown macules (2mm-8mm) all over the body in non/sun exposed areas. Biopsy show single and clustered atypical melanocytes at the DE junction w/o upward migration, linear fibrosis of the superficial dermis, and sparse inflammatory infiltrate in dermis.

Answer 1

dysplastic nevus syndrome

*melanocytes

Question 2

increased pigment in basal keratinocytes

Answer 2

ephelis

Question 3

Tumor on legs of middle-age and young women. Asx or tender. indolent behavior. Hx of antecedent trauma. Factor 13a-positive dermal dendritic cells.

Answer 3

B9 Fibrous Histiocytoma (dermatofibroma)

Question 4

Firm, tan to brown papules. B9, spindle shaped cells. Many cases demonstrate a peculiar form of overlying epidermal hyperplasia, characterized by downward transformation….

Answer 4

B9 Fibrous Histiocytoma (dermatofibroma)

Question 5

may suddenly appear in large numbers as part of a paraneoplastic syndrome (leser trelate sign) due to stimulation of keratinocytes by transforming GF-alpha produced by tumor cells of the GI tract. FGFR3

Answer 5

seborrheic keratoses

Question 6

Flat, coin like, waxy plaques

Answer 6

seborrheic keratoses

Question 7

scaly, red brown patches

Answer 7

Mycosis Fungoides (CTCL)

Question 8

CDNK2A gene LoF (skin)

Answer 8

dysplastic nevi

Question 9

“peculiar linear fibrosis” (skin)

Answer 9

dysplastic nevi

Question 10

BRAF (skin)

prognosis?

Answer 10

sporadic melanoma

better - have monoclonal antibody to BRAF

Question 11

FGFR2 (skin)

Answer 11

SK’s and acanthosis nigricans (PCOS), achondropalsia

Question 12

MSH2, MLH1 (skin)

Answer 12

HNPCC and Muir Torre

Question 13

SHH (skin)

Answer 13

BCCa

Question 14

34year old white F for moles that have become darker, larger, more numerous.
PE: chloasma and linea nigra present. 15 pigmented macules present; all

Answer 14

achrocordons (skin tag/fibroepithelial polyp)

Question 15

Change in size and color during pregnancy

Answer 15

achrocordons (fibrepithelial polyp/skin tag)
melanocytic nevi (pigmented nevus, mole)

Question 16

genetic abnormaltiy of myxoid

Answer 16

…??

Question 17

eccrine poroma location

Answer 17

sweat glands

Question 18

pilar cyst or wen

Answer 18

epithelial or follicular inclusion cyst

Question 19

69 year old farmer with male baldness with lesion on top of his head.
PMH: orthotopic cardiac transplant at 60yo for hypertrophic cardiomyopathy
PE: well healed scars from prior sclap excicional biopsies. Red-tan, nodular and centrally ulcerated 14cm lesions on scalp. SCattered red/tan hyperkaratotic lesions on face, scalp, arms.
Biopsy of nodular mass reveals atypical polygonal cells with intercellular bridges extending into dermis and involving lymphtics.

Answer 19

Squamous cell carcinoma

Question 20

risk factors for SCCa

Answer 20

• immunosuppression (transplant)

- ionizing radiation (**UV light –> DNA damage)

Question 21

urticaria pigmentosa, darier sign (wheal/erythema/edema), dermatographism (“skin drawing”)

Answer 21

mastocytosis

Question 22

KIT

Answer 22

Mastocytosis, AML, GIST, hematologic diseases

Question 23

Characteristic storiform (swirling) alignment of spindle cells (skin)

Answer 23

Dermatofibrosarcoma protuberans (DFSP)

Question 24

TP53 (skin)

Answer 24

squamous cell carcinoma

Question 25

Precursor lesions to SCCa skin

Answer 25

``` AK's (TLRs) Epidrermodysplasia verriciformis (HPV) Xeroderma pigmentsum (pyrimidine dimers) Lichen planus (chronic inflammation) ```

Question 26

SEE MOLECULAR SLIDE FROM DOBSON | SEE SYNDROMES/SIGNS FROM DOBSON

Answer 26

FOR CHAPTER 25!!! | FOR CHAPTER 25

Question 27

19year old female for multiple macules. - PMH: ovarian fibroma with ascites and hydrothorax. - PE: multiple pearly papules, some appear pigmented. Vascular telangiectasia. Some erythematous and ulcerated. Multifocal islands of basophilic cells in a mucinous matrix. Periphery palisading. - What does she have? - What germline mutation?

Answer 27

PTCH (Nevoid BCCa Syndromes or Gorlin syndrome)

Question 28

table 25-3: NBCCasyndrome cowden Femailial

Answer 28

see table

Question 29

**BRAF (skin) | LoF CDKN2A

Answer 29

melanocytic lesions

Question 30

FGFR3 (two entities)

Answer 30

seborrheic keratosis | acanthosis nigricans

Question 31

FGFR3, IGFR1, TGF-alpha

Answer 31

acanthossi nigricans

Question 32

PTEN | trichilmmomas and breast/endometrial/other Cancers

Answer 32

cowden

Question 33

CYLD

Answer 33

brooke spiegler

Question 34

CTNNB1

Answer 34

pilomatricoma

Question 35

***TLRs

Answer 35

actinic keratosis (horns!)

Question 36

TP53

Answer 36

SCCa

Question 37

**SHH/PTCH

Answer 37

BCCa

Question 38

**PDGF-beta | COL1A1

Answer 38

Dermofibrosarcoma protuberans

Question 39

KIT

Answer 39

mastocytosis

Question 40

TNF

Answer 40

psoriasis (and HLA-C)

Question 41

TLR2

Answer 41

rosacea

Question 42

dysplastic nevus syndrome has high risk of ___.

Answer 42

melanoma

Question 43

Leser Trelat sydrome is...

Answer 43

sudden appearance of Seborrheic keratosis and paraneoplastic syndrome is

Question 44

Birt-Hogg-Dube syndrome is...

Answer 44

fibroeptihelial polyps and perifollicular mesenchymal tumors

Question 45

trichoepithelioma and cylindroma (CYLD mutation) is ___syndrome

Answer 45

brooke-spigler syndrome

Question 46

Muir-Torre syndrome is what two thigng?

Answer 46

internal malignancy (Hereditary NonPolyposis Colorectal Carcinoma syndrome) and sebaceous adenoma

Question 47

BCCa's and other tumors (ovarian fibroma, medulloblastoma)

Answer 47

Gorlin/NBCCS/Basal cell nevus

Question 48

mycosis fungoides with blood involvement = ___sundrome

Answer 48

Sezary sydrome

Question 49

dermal edema and erythema (wheal) after rubbing lesion in mastocytosis is ___sign

Answer 49

darier sign

Question 50

febrile form of erythema multiforme

Answer 50

SJS

Question 51

ausptiz sign

Answer 51

punctate bleeding in psoriasis when scale is lifted