L6 - Derm Pathology Flashcards

1
Q

What are the signs of dysplasia?

A
Disordered growth 
Loss in uniformity of the individual cells 
Loss of architectural orientation 
Pleomorphism 
Hyperchromatic 
Increased mitosis (normal and abnormal)
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2
Q

Which mutations occur in melanocytic nevi?

A

Activating mutations in components of the RAS or BRAF signaling pathway

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3
Q

What are junctional nevi?

A

Nests at dermoepidermal junction

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4
Q

What are compound nevi?

A

Nests or cords grow into underlying dermis

Nests in epidermis and dermis

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5
Q

Which genes are involved in dysplastic Nevus syndrome?

A

CDKN2A or CDK4 genes

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6
Q

Dsyplastic nevi often acquire activating mutations in which genes?

A

NRAS and BRAF genes

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7
Q

What are the ABCDEs of melanoma?

A
Asymmetry 
Irregular Borders 
Variegated Color 
Increasing Diameter 
Evolution/change over time (esp rapid) 
Any pigmentated legion with diameter >6mm, any change, itching or pain
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8
Q

What characteristics are associated with a favorable prognosis for melanoma?

A
Thinner tumor depth 
NO (<1 per mm) mitosis 
Brisk tumor infiltrating lymphocyte response 
NO regression 
Lack of ulceration
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9
Q

What is the Leser Trelat sign?

A

Paraneoplastic syndrome
Sudden appearance of large numbers of Seb Ks
Associated with carcinomas of GI tract

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10
Q

Describe nevoid BCC syndrome

A

AD inheritance
PTCH gene
Developmental patterning gene/ multiple BCC
Medulloblastoma, jaw cysts

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11
Q

Describe Cowden syndrome

A

AD inheritance
PTEN
Lipid phosphatase/benign follicular appendage tumors (tichilemmomas); internal adenocarcinoma (often breast or endometrial)

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12
Q

Describe familial melanoma syndrome

A

AD inheritance

CDKN2

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13
Q

Describe Muir-Torre syndrome

A

AD inheritance
MLH1/MSH2
Involved in DNA mismatch repair/sebaceous neoplasia
Internal malignancy (colon and others)

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14
Q

Describe xeroderma pigmentosum

A

AR inheritance
XPA gene
Mutation in NER
Increased risk for SCC, melanoma and non melanoma skin cancers

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15
Q

What is actinic chelitis?

A

Similar lesions to actinic keratosis on the lips

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16
Q

What is the second MC tumor arising in sun exposed sites?

A

Squamous cell carcinoma

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17
Q

What signaling pathway and gene is affected in BCC?

A

Unbridled hedgehog signaling

PTCH gene

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18
Q

What are pearly papules associated with?

A

BCC

Also have telangiectactic vessels

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19
Q

What translocation is associated with dermatofibrosarcoma protuberans (DFSP)?

A

Translocation collagen 1A1 (COL1A1) and PDGFB

Promoting tumor cell growth

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20
Q

The honeycomb pattern is a pathologic characteristic of which condition?

A

DFSP
It is a deep extension from the dermis into the SubQ fat
Also called Swiss cheese appearance on histo slides
Also associated with storiform (swirling spindle cells)

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21
Q

What is sezary syndrome?

A

Erythroderma, diffuse erythema and scaling of entire body surface ‘

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22
Q

What are sezary cells?

A

Markedly folded nuclear membrane, hyper convoluted or cerebriform contour
Band like aggregates within the superficial dermis
Pautrier microabscesses (small clusters of cells in the epidermis)

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23
Q

What is solitary mastocytoma?

A

Usually young kids
Pink-tan brown nodule
+/- pruritic or blister formation

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24
Q

What point mutation is associated with mastocytosis?

A

KIT receptor tyrosine kinase —> mast cell growth and survival

25
What is ichthyosis vulgaris?
AD or acquired Congenital ichhtyosiform erythroderma (AR) Lamellar ichthyosis (AR) X lined ichthyosis Most become apparent either at or around the time of brith Acquired (noninherited) variants also exist
26
What are characteristics of acute inflammatory lesions?
Last from days to weeks and are characterized by inflammatory infiltrates (usually composed of lymphocytes and macrophages rather than neutrophils), edema and variable degrees of epidermal, vascular, or subcutaneous injury
27
What are the characteristics of chronic lesions?
Persist for months to years and are often associated with changes in epidermal growth (atrophy or hyperplasia) or derma fibrosis
28
What are wheals?
Pruritic edematous plaques
29
What is angioedema?
Closely related to urticaria | Edema of the deeper dermis and subQ fat
30
What is the mechanism associated with urticaria?
Localized mast cell degranulation —> dermal micro vascular hyper permeability Ag induced release of vasoactive mediators from mast cells
31
Describe macro urticaria
Small pruritic plaques to large edematous plaques | Individual lesions may coalesce to form annular, linear or acriform configurations
32
Describe micro urticaria
Sparse superficial perivenular infiltrate of mononuclear cells and rare neutrophils +/- eosinophils Collagen bundles more widely spaced than normal skin due to edema Dermal lymphatic may be dilated due to absorption of edema fluid
33
Which Ab is associated with urticaria?
IgE
34
What is the Koebner phenomenon?
Induce psoriatic lesions in susceptible pts by local trauma Starts self perpetuating local inflammatory process Also seen with Lichen planus
35
What is follicular lipping and what is it associated with?
Mounds of parakeratosis containing neutrophils and serum are present at the Ostia of hair follicles Associated with seb dermatitis
36
Describe the lesions seen in seb dermatitis
Individual lesions are macules and papules on an erythematous yellow often greasy base typically in association with extensive scaling and crusting Fissures may also be present particularly behind the ears
37
What is the common clinical expression of seb dermatitis of the scalp?
Dandruff
38
What are the 6 P’s of lichen planus?
Pruritic, purple, polygonal, planar, papules and plaques
39
Bullous diseases produce what?
Dramatic lesions which be fatal if untreated Blisters occur at different levels of the skin Histologic assessment essential for accurate diagnosis
40
What are the histologic features seen with pemphigus vulgaris?
Tombstone appearance Fishnet like appearance Foliaceus (more superficial)
41
What is epidermolysis bullosa?**
Group of non inflammatory blistering diagnoses Inherited defects in structural proteins Blisters at sites of pressure, rubbing or trauma at or soon after birth Electron microscopy to differentiate between
42
What is porphyria?**
Group of inborn or acquired disturbances in porphyrin metabolism Porphyrins are pigments normally present in Hb, myoglobin and cytochromes Urticaria and vesicles associated with scarring Exacerbated by sunlight
43
What are open comedones?
Central black (oxidation of melanin) keratin plug
44
What are closed comedones?
Keratin plug trapped beneath epidermal surface | Potential for follicular rupture and inflammation
45
What is acne conglobate?
Severe variant of acne vulgaris | Sinus tract formation and dermal scarring
46
What are other types of panniculitis?
Weber Christian disease (relapsing febrile modular panniculitis) Factitial panniculitis T cell lymphoma SLE
47
What is Weber Christian disease?
Rare form of lobular nonvasculitic panniculitis Children and adults Crops of erythematous plaques or nodules predominantly on LE Created by deep seated Foci of inflammation containing aggregates of foamy macrophages with lymphocytes, neutrophils and giant cells
48
What is factitial panniculitis?
Form of secondary panniculitis caused by self inflicted trauma or injection of foreign or toxic substances
49
What are histologic characteristics associated with verruca vulgaris?
Verrucous or papillomatous epidermal hyperplasia (spire like epidermal hyperplasia) Koilocytosis (cytoplasmic vacuolization)
50
List superficial fungal infections
Tinea capitis, tinea Barbae, tinea corporis, tinea cruris, tinea pedis (athletes foot), tinea versicolor
51
What is tinea capitis?
Usually kinds rarely seen in infants and adults Dermatophytosis of the scalp Asymptomatic, often hairless patches of the skin with mild erythema, crust formation and scaling
52
What is tinea barbae?
Dermatophyte infection of the beard Adult men Relatively uncommon disorder
53
What is tinea corporis?
Common superficial fungal infection of the body surface All ages but esp kinds Predisposing factors include excessive heat and humidity, exposure to infected animals, chronic dermatophytosis of the feet or nails Most common: expanding, round, slightly erythematous plaque with elevated scaling border
54
What is tinea cruris?
Inguinal areas of obese men during warm weather Heat, friction and maceration all predispose to its development The infection usually first appears on the upper inner thighs as moist, red patches with raised scaly borders
55
What is tinea pedis (athletes foot)?
Affects 30-40% of the population at some time in their lives Diffuse erythema and scaling often initially localized to the web spaces Most of reaction due to bacterial superinfection and not directly related to the primary dermatophytosis Can infect the nails (onychomycosis) —> discoloration, thickening and deformity of the nail plate
56
What is tinea versicolor?
``` Upper trunk, highly distinctive in appearance Malassezia furfur (a yeast not a dermatophyte) the lesions consist of groups of macules of varies size and color with a fine peripheral scale ```
57
What is panniculitis?
Inflammatory reaction of the SubQ adipose tissue
58
What are intradermal nevi?
No epidermal nests | Usually older lesions