Bafl Exam 2: Cysts

Front 1

List the stratified squamous cysts?

Back 1

aw girl, DEM TVS! PPPP

Dermoid

Epidermoid

Milia

Trichilemmal

Vellus

Steatocystoma

Proliferating epidermoid

Proliferating trichilemmal

Pits

Pigmented follicular

Pilonidal

Front 2

List the non stratified squamous cysts?

Back 2

Hydrate your throat and genitals!

Hydrocystoma (apocrine and eccrine)

Throat- (ish) bronchogenic, thyroglossal, branchial

Genitals- cutaneous ciliated, median raphy, omphalomesenteric

Front 3

List the non epithelial cysts?

Back 3

Mucocele

Digital mucous cyst

Ganglion cyst

Pseudocyst of the auricle

Front 4

Which cysts are derived from the follicular infundibulum? What is the follicular infundibulum?

Back 4

Follicular infundibulum is the area between the sebaceous duct and the surface of the hair

Epidermoid, milium, pigmented follicular, vellus hair

Front 5

Most common cutaneous cyst? Where does it come from?

Back 5

Epidermoid cyst aka epidermal cyst, epidermal inclusion cyst, infundibular cyst and keratin cyst

Develops from infundibular area

Front 6

Multiple epidermoid cysts are seen in what syndromes?

Back 6

GORLIN'S

GARDNER'S

(also seen in scrotal calcinosis and significant acne)

Front 7

Buzz word: multiple lesions of congenital hypertrophy of the retinal pigmented epithelium

Back 7

CHRPE! Multiple are associated with Gardner's syndrome aka familial adenomatous polyposis

birds CHRPE in the garden

Front 8

Gene defect in Gardner's syndrome?

Back 8

AD defect in APC gene- regulates B catenin

Front 9

See epidermoid cysts, fibromas, osteomas together. What else do you see?

Back 9

Gardner's syndrome- will also see GI polyposis --> colorectal cancer

May also see odontomas and supranumerary teeth

This is a defect in the APC gene (dysregulation of B catenin)

Front 10

AD, PTCH1 mutation best decribes..

Back 10

Basal Cell Nevus Syndrome (Gorlins)

Front 11

Multiple BCC, palmoplantar pits, epidermoid cysts, which mutation?

Back 11

Gorlin's syndrome- AD mutation of PTCH 1 gene

Front 12

What is seen in Gorlin's syndrome?

Back 12

BCC, plamoplantar pits, epidermoid cysts, jaw cysts, frontal bossing, bifid ribs, kyphoscoliosis, calcificatino of the falx cerebri, medulloblastoma

Front 13

Ovarian fibomas, epidermoid cysts, calcification of the falx cerebri?

Back 13

Gorlin's syndrome

Front 14

Path of epidermoid cysts?

Back 14

Stratified squamous epithelium, +granular layer, cavity filled with keratin

Front 15

HPV infected epidermoid cysts?

Back 15

Verrucous cysts- HPV 60

Front 16

Proliferating epidermoid cysts are most common in what locations?

Back 16

PELVIC/ANOGENITAL (36%)

mc in men

Front 17

DDX for this?

Back 17

Pilar sheath cyst- which is what this is, MC on upper lip of adults, will see bulbous proliferations pushing into surrounding dermis on path

DDX must include: Dilated pore of Winer: These contain a larger cystic space which is surrounded by radiating thin strands of epithelium as opposed to the lobular arrangement of pilar sheath acanthoma

Front 18

Milia on the hard palate?

Back 18

Bohn's nodules

(hard = boner)

Front 19

Milia on the gums?

Back 19

Epstein's pearls

Front 20

Milia behind the ear on a red base? What is this associated with?

Back 20

Milia en plaque, associated with lupus, or as result of trauma

Front 21

Multiple milia can be associated with?

Back 21

Marie-Unna hypotrichosis (hereditary trichodysplasia- AD sparse hair at birth, wirey growth during childhood, loss of hair in adolescence)

Oral-facial-digital syndrome type 1(mutation of OFD1 causes oral, facial, digital malformation in the setting of polycystic kidney disease)

Rombo and Bazex syndrome (BCC)

Front 22

What are the features on path of a Trichilemmal cyst?

Back 22

1. abrupt keratinization

2. no granular layer

3. swollen and pale keratinocytes

4. stratified squamous epithelium

5. calcification/cholesterol clefts

Front 23

Where do pilar cysts originate?

Back 23

Isthmus

Front 24

Isthmus cysts?

Back 24

Pilar/Wen/Isthmus-catagen

Front 25

Proliferating trichilemmal cysts, who grows em and are they bad?

Back 25

Usually women in their 60s on their scalp, usually benign, but can grow and become metastatic (variant of SCC?)-- very locally invasive, can grow to 25cm

Front 26

Relationship between proliferating trichilemmal cysts and KID syndrome? Mutation in KID?

Back 26

KID syndrome- keratosis, ichthyosis, deafness

malignant proliferating pilar tumors may occur in young adults and be fatal

KID- connexin 26 (kids always played ConnectFour!)

Front 27

'Rolls and scrolls'

Back 27

Proliferating trichilemmal cyst

Front 28

Eruptive vellus hair cysts may be associated with...

Back 28

Steatocystoma multiplex-

Pachyonychia congenita type 2-

Front 29

Where are steatocystomas typically found?

Back 29

chest, axillae, groin

Front 30

Steatocystoma multiplex is a mutation in which gene?

Back 30

AD mutation in keratin 17-- identical to mutation found in pachyonychia congenita type 2

Front 31

Pachyonychia congenita I vs pachyonychia congenita II, what are the mutations and findings?

Back 31

PCI- Jadassohn Lewandowsky, mutation in K6a and 16, associated with subungual hyperkeratosis, leukokeratosis, palmoplantar calluses

PCII- Jackson Lawler, AD mutation in K6b and 17, associated with natal teeth, steatocystona multiplex, eruptive vellus hair cysts, epidermoid cysts

Front 32

Which cysts are associated with Gorlins?

Back 32

epidermoid and cutaneous keratocysts

Front 33

What must we be concerned about in dermoid cysts?

Back 33

Dermoid cysts are present in infants along embryonic fusion plane (mc eye), if they need to be removed, should get imaging first to r/o neural heterotopias (barbell)

Front 34

Histology of a dermoid cyst?:

Back 34

Stratified squamous cyst, + granular layer, can have hair, sebacous lobules, eccrine glands, apocrine glands, smooth muscle

Front 35

How does the ear form embryologically?

Back 35

3 tubercles from first two branchial arches

Front 36

How do ear pits arise? What are they associated with?

Back 36

Preauricular cyst from defective embryologic fusion with epithelial entrapment, a/w

1. branchio-otic syndrome --> deafness

2. branchil-oto-renal dysplasia --> renal abnormalities

Front 37

What is the follicular occlusion tetrad?

Back 37

Acne conglobata, hidradenitis suppurativa, pilonidal disease, dissecting cellulitis

Front 38

Histologic differences between apocrine and eccrine hidrocystomas?

Back 38

Apocrine hidrocystomas- adenomas of apocrine sweat glands usually on lower eyelid, unilocular or multilocular, lined by one to several layers; Clinically this lesion will be SOLITARY

Eccrine hidrocystomas- adenomas of eccrine sweat glands, unilocular always, lined by two layers of flattened cuboidal epithelium always; clinically there will be multiple lesions that enlarge in the heat

Front 39

Cysty/pitty lesion at suprasternal notch?

Back 39

Bronchogenic cyst- fistulous tract, found at birth, respiratory epithelium (pseudostratified ciliated columnar epithelium with goblet cells) sequestered during development

Front 40

Midline neck cyst , moves with swallowing?

Back 40

Thyroglossal duct cyst- arise from remnants of the thyroglossal duct, will see thyroid follicles on path

Front 41

Cyst on anterior border of SCM, noticed age 25?

Back 41

Branchial cleft cyst- will see lymphoid tissue on path

Front 42

Cyst can be found either on LE of young women or on labia majora?

Back 42

cutaneous ciliated cyst

Front 43

Cyst found on young men, ventral aspect of the penis?

Back 43

median raphe cyst

Front 44

This cyst shares same developmental abberation as a Meckels diverticulum?

Back 44

Omphalomesenteric duct cyst- developmental defect in the closure of the omphalomesenteric duct

Front 45

Kid leaking urine from umbilicus?

Back 45

Urachal cyst

Front 46

contains what material inside?

Back 46

Mucocele- mc on lower labial mucosa, mucinous material --> SIALOMUCIN

**superficial mucoceles are mc on retromolar pad, posterior buccal mucosa and soft palate

Front 47

Which genodermatosis is associated with multiple hidrocystomas?

Back 47

Schopf-Schulz-Passarge

ectodermal dysplasia, multiple eyelid apocrine hidrocystomas, palmoplantar keratoderma, hypodonia, hypotrichosis, nail dystrophy

Front 48

Multiple types of these cysts may be found in these 2 genodermatoses?

Back 48

Gardner syndrome

Gorlin's syndrome

Front 49

2 associated findings?

Back 49

Dermoid cyst:

hearing loss (branchio-otic syndrome)

renal abnormalities (branchio-oto-renal dysplasia)

Front 50

Histological features of steatocystomas? Where do they come from?

Back 50

TRUE SEBACEOUS CYSTS! Arise from sebaceous duct.

see stratified squamous epithelium, no granular layer, siiregular corrugated cuticle ('shark tooth')

Front 51

what age does this present?

Back 51

second or third decade of life