Dermatopathology I - Fung Flashcards

1
Q

What are the 3 layers of skin?

A
  1. epidermis - contains keratinocytes
  2. dermis - filled with collagen for support and adnexal structures
  3. sub Q tissue - importan for shock absorbance, contains fat
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2
Q

What are the layers of the epidermis?

A
  1. stratum corneum - stratified squamous cells (keratinocytes)
  2. stratum lucidum - only on palms and soles of feet
  3. stratum granulosum - dark blue layer, contains granules that combine with tonofibrils to form keratin
  4. stratum spinosum - where cells mature, contains polyhedral keratinocytes that produce tonofibrils that will eventually form desmosomes
  5. stratum germinativum
  6. stratum basale - where cells are formed
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3
Q

What are desmosomes?

A

Intracellular bridges.

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4
Q

What happens to keratinocytes as they mature?

A

They lose their nucleus and cytoplasm and extrude keratin.

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5
Q

What types of cells are located in the epidermis?

A
  1. keratinocyes - produce keratin
  2. langerhans cells - immune function for skin
  3. melanocytes - protect skin from UV rays
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6
Q

What does the basement membrane do?

A

It connects the epidermis to the dermis.

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7
Q

Name two types of glands found in the skin.

A
  1. apocrine sweat glands - occur mainly in the axilla and groin - do not secrete anything
  2. eccrine glands - secrete sweat and function in thermal regulation
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8
Q

Describe a macule.

A
  1. circumscribed
  2. flat lesion of less than 5mm
  3. distinguished from surrounding skin by color
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9
Q

Describe a patch.

A
  1. circumscribed
  2. flat lesion of greater than 5 mm
  3. distinguished form surrounding skin by color
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10
Q

Describe a papule.

A
  1. elevated
  2. dome-shaped or flat topped lesion
  3. less than 5 mm
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11
Q

Describe a nodule.

A
  1. elevated
  2. dome shaped or flat topped lesion
  3. greater than 5 mm
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12
Q

Describe a plaque.

A
  1. elevated

2. flat topped lesion of greater than 5 mm

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13
Q

Describe a pustule.

A

Discrete pus filled raised lesion.

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14
Q

Describe scale.

A
  1. dry
  2. horny and plate-like excrescence
  3. usually the result of imperfect cornification
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15
Q

Describe a vesicle.

A
  1. fluid filled
  2. raised lesion
  3. less than 5mm
  4. also called a blister
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16
Q

Describe a bulla.

A
  1. fluid filled
  2. raised lesion
  3. greater than 5 mm
  4. also called a blister
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17
Q

Describe an excoriation.

A

Traumatic lesion breaking the epidermis and causing a raw linear area or deep scratch.

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18
Q

Describe a wheal.

A
  1. itchy
  2. transient
  3. elevated lesion with variable blanching and erythema
  4. dermal edema
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19
Q

Describe lichenification.

A

Thickened rough skin, usually the result of repeated rubbing.

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20
Q

What is ancanthosis?

A

Diffuse epidermal hyperplasia. Seen a lot in chronic inflammatory dermatoses.

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21
Q

What is dyskeratosis?

A

Abnormal, premature keratinization within cells below the stratum granulosum. A malignant process. Can see lots of pink in the epidermis.

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22
Q

What is hypergranulosis?

A

Hyperplasia of the stratum granulosum.

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23
Q

What is hyperkeratosis?

A

Thickening of the stratum corneum.

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24
Q

What is Lentingenous?

A

A linear pattern of melanocyte proliferation within the epidermal basal cell layer.

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25
Q

What is papillomatosis?

A

Surface elevation caused by hyperplasia and enlargement of contiguous dermal papillae.

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26
Q

What is parakeratosis?

A

Keratinization with retained nuclei in the stratum corneum.

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27
Q

What is spongiosis?

A

Intracellular edema of the epidermis.

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28
Q

Describe acute inflammatory dermatoses.

A
  1. last from days to weeks

2. characterized by lymphocytic and macrophage inflammatory infiltrate and edema

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29
Q

Describe chronic inflammatory dermatoses.

A
  1. lasts from months to years
  2. associated with changes in epidermal growth like atrophy or hyperplasia or dermal fibrosis
  3. the skin is roughened due to excess or abnormal scale formation and shedding
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30
Q

Name some acute inflammatory dermatoses.

A
  1. urticaria
  2. acute eczematous dermatitis
  3. erythema multiforme
31
Q

Name some chronic inflammatory dermatoses.

A
  1. psoriasis
  2. seborrheic dermatitis
  3. lichen planus
32
Q

What is urticaria?

A
  1. common disorder
  2. characterized by localized mast cell degranulation and dermal microvascular hyper permeability
  3. patients present with pruritic edematous plaques or wheals
  4. angioedema is a related condition with edema of the deeper dermis an sub Q fat
  5. can sometimes be chronic
33
Q

What will urticaria look like microscopically?

A

Will see normal epidermis and edema and sparse inflammation in the papillary dermis.

34
Q

What are the causes of urticaria?

A
  1. immunologic mechanisms
  2. non-immunologic mechanisms
  3. physical stimuli
  4. skin contact
  5. small vessel vasculitis
35
Q

What is the treatment of uritcaria?

A
  1. avoidance of specific allergens
  2. oral H1 antagonists
  3. epinephrine
36
Q

What is acute eczematous dermatitis?

A
  1. one of the most common skin disorders
  2. pathogenesis is T-cell mediated inflammatory reactions (Type IV hypersensitivity)
  3. Can be subdivided into 5 categories
37
Q

What are the 5 subcategories of acute eczematous dermatitis?

A
  1. allergic contact dermatitis
  2. atopic dermatitis
  3. drug-related eczematous dermatitis
  4. photoeczematous dermatitis
  5. primary irritant dermatitis
38
Q

What does acute eczematous dermatitis look like microscopically?

A
  1. spongiosis
  2. confluent parakeratosis
  3. can have formation of intraepidermal vesicles
39
Q

What are the causes of acute eczematous dermatitis?

A

Can be broadly categorized as:

  1. inside - reaction to an internal circulating antigen
  2. outside - reaction from external application of antigen
40
Q

What is the treatment of eczematous dermatitis?

A
  1. removal of the offending substance

2. topical steroids

41
Q

Describe erythema multiforme.

A
  1. self-limited hypersensitivity reaction
  2. associated with infection from viruses (Herpes simplex I), bacteria (mycoplasma, leprosy, typhoid) and fungi (histoplasma, coccidiodes)
  3. exposure to drugs can also cause (sulfonamides, penicillin, barbiturates, salicylates, antimalarials)
  4. associated with cancer and collagen vascular disease
42
Q

What does erythema multiforme look like macroscopically?

A

It can present with lesions of various sizes and shapes but the classic lesions are targatoid plaques with crust in the center.

43
Q

What does erythema multiforme look like microscopically?

A
  1. spongiosis
  2. dermal edema
  3. perivascular lymphocytes
  4. necrotic keratinocytes
44
Q

What are two syndromes that are associated with erythema multiforme?

A
  1. Stevens-Johnson syndrome - febrile form, extensive skin involvement, possible involvement of oral mucosa, conjunctiva, urethra, genitals and perianal areas, can lead to sepsis and is mainly seen in children.
  2. toxic epidermal necrolysis - characterized by diffuse necrosis and sloughing of cutaneous and mucosal epithelial surfaces, pt resembles burn victim clinically
45
Q

Describe Psoriasis.

A
  1. common disorder affecting 1-2% of the US population
  2. chronic inflammatory dermatosis that results from interactions of genetic and environmental factors
  3. associated with HLA-C
  4. results from activated T cells in the skin stimulating the secretion of cytokines and growth factors that induce keratinocyte proliferation
  5. 15% of patients have associated arthritis
46
Q

What does Psoriasis look like macroscopically?

A

Like red, scaly plaques.

47
Q

What does Psoriasis look like microscopically?

A
  1. spongiosis
  2. acanthosis
  3. lymphocytes within epidermis and dermis
  4. confluent parakeratosis
  5. may present with micro hemorrhages on the skin due to papillary dermis being next thinned epidermis - called Auspitz’s sign
  6. may have monroe abcesses or spongioform pustules within the spongioform dermis
48
Q

What are the treatments for Psoriasis?

A
  1. topical steroids
  2. intralesional steroid injection
  3. UVB and tar
  4. methotrexate
  5. cyclosporine
  6. soriatane
49
Q

Describe seborrheic dermatisis.

A
  1. common chronic inflammatory dermatosis that affects up to 5 % of the general population
  2. involves regions with density of sebaceous glands such as:
    scalp
    forehead
    external auditory canal
    retroauricular area
    nasolabial folds
    presternal area
50
Q

What are the causes of Sebhorreic dermatitis?

A
  1. increased sebum production
  2. colonization of the skin by Malasseria
  3. severe form seen in HIV infection
51
Q

What does seborrheic dermatitis look like macroscopically?

A

Like erythematous plaques.

52
Q

what are the treatments for sebhorreic dermatitis?

A
  1. frequent washing of affected areas with antisebhorreic soaps
  2. topical steroids
  3. anti-yeast medications
  4. oral antifungals
53
Q

Describe Lichen planus.

A
  1. self-limited condition most commonly resolving spontaneously 1-2 years after onset
  2. resolution of lesions may leave post inflammatory hyper pigmentation
  3. squamous cell carcinoma may develop in oral lesions
  4. characterized by the 4 P’s - pruritic, purple, polygonal, planar, papules and plaques
54
Q

What does lichen planus look like macroscopically?

A
  1. appears like white film on tongue
  2. erythematous macule with white striations on skin
  3. the striations on the tongue and skin are called Wickham’s striae and are due to hypergranulosis
55
Q

What does lichen planus look like microscopically?

A
  1. will see lichenoid inflammation - thick band of lymphocytic infiltration at the epidermal-dermal border
  2. hyperkeratosis
  3. hypergranulosis
56
Q

What are the treatments for lichen planus?

A
  1. topical steroids
  2. intralesional steroids
  3. systemic steroids
  4. azathioprine
  5. cyclosporine
  6. light therapy
57
Q

Many conditions exist in which blisters are a feature. What is it called when blisters are the primary and most distinctive feature?

A

These types of disorders are called Bullous disorders. The causes are both inflammatory and non-inflammatory.

58
Q

Where do blisters form?

A

Blisters can form in several layers of the skin.

  1. subcorneal blisters form in the stratum corneum
  2. suprabasal blisters form above the stratum basal
  3. subepidermal blisters form below the stratum basal at the level of the basement membrane
59
Q

What are some proteins that are involved in blister formation?

A

Desmoglein 1 and 3.

60
Q

What is pemphigus?

A
  1. an inflammatory blistering disorder caused by autoantibodies that result in the dissolution of intracellular attachments within the epidermis and mucosal epithelium
  2. IgG autoantibodies to desmoglein 1 and 3 disrupt intercellular adhesions of desmosomes.
61
Q

What are the different variants of Pemphigus?

A
  1. Pemphigus vulgaris - most common, forms supra basal blisters
  2. Pemphigus vegetans - forms plaques around the groin area
  3. Pemphigus foliaceus - forms sub corneal blisters
  4. Pemphigus erythematus - less severe form of Pemphigus foliaceus
  5. Paraneoplastic pemphigus - associated with cancer
62
Q

If you did an immunofluorescence assay on a Pemphigus foliaceus blister what would you see?

A

You would see a ‘swiss cheese’ pattern of fluorescence at the sub corneal level corresponding to IgG antibodies.

63
Q

How is pemphigus treated?

A

Treated with immunosuppressive agents to decrease the titers of pathogenic antibodies.

64
Q

What is bullous pemphigoid?

A
  1. a blistering disorder caused by autoantibodies directed to the proteins that bind basal keratinocytes to the basement membrane
  2. antibody deposition occurs in a linear pattern at the dermoepidemral junction
  3. the proteins- called BPAGs -are a part to eh hemidesmosomes that link basal keratinocytes to the basement membrane
65
Q

What does bullous pemphigoid look like macroscopically?

A

Diffuse blisters that don’t break when touched (unlike blisters in pemphigus) and heal without scarring.

66
Q

What does bullous pemphigoid look like microscopically?

A
  1. inflammatory infiltrate in blister
  2. epidermis is raised off of dermis due to the sub-epidermal blister
  3. on immunofluorescence you would see a linear pattern of IgG on surface of blister
67
Q

What is the treatment for bullous pemphigoid?

A
  1. topical steroids
  2. systemic steroids
  3. methotrexate
  4. azathioprine
68
Q

What is dermatitis herpetiformis?

A
  1. a rare disorder characterized by urticaria and grouped vesicles
  2. has a strong associated with HLA-B8, HLA-DR3 and HLA-DQw2
  3. genetically predisposed individuals develop IgA antibodies to dietary gluten
  4. these antibodies cross react with reticulin which is a component of the anchoring fibrils that attaches the epidermal basement membrane to superficial papillary dermis
  5. injury results in sub epidermal bisters - see blisters most often on elbows and knees
69
Q

What does dermatitis herpetiformis look like microscopically?

A
  1. will see subepidermal blisters
  2. lymphocytic infiltrate in blister
  3. IgA in peaks of papillary layer in immunofluorescence
70
Q

How is dermatitis herpetiformis treated?

A
  1. sulfapyridine

2. dapsone

71
Q

What is epidermolysis bullosa?

A
  1. a non-inflammatory blistering disorder caused by an inherited defect in structural proteins that cause mechanical instability to the skin
  2. clinical manifestations occur soon after birth with blister formation at sites of pressure
  3. there are 4 types
72
Q

What are the 4 types of epidermolysis bullosa?

A
  1. simplex - autosomal dominant inheritance of defects in keratin 14 or keratin 5 resulting in defects in the basal cell layer of the epidermis - see supra basal blisters
  2. junctional - autosomal recessive inheritance of defects in laminin, a protein at the laminal lucida which binds to both hemidesmosomes and anchoring filaments - blisters are subepidermal at level of lamina lucida
  3. dystrophic - autosomal dominant or recessive inheritance of defects in type VII collagen which is a major component of the basement membrane anchoring fibrils - blisters are sub epidermal at level of lamina dense, blisters leave scars
  4. mixed
73
Q

If you did an immunofluorescence assay of a sample of epidrmolysis bullosa what would you see?

A

You would see IgG on the floor of the blister.