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Pathology Unit 2 > Ch 24: Skin: BM, Vascular, Acne > Flashcards

Ch 24: Skin: BM, Vascular, Acne Flashcards

1
Q

Heterogeneous group of disorders loosely bound by their hereditary nature which form blisters at sites of minor trauma and are usually noted at birth or shortly after

A

epidermolysis bullosa (EB)

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2
Q

Group of autosomal recessive and autosomal dominant EB attributed to cytokeratin intermediate filament gene mutations which involve cytolysis of basal keratinocytes and healing without scar formation

A

Epidermolytic EB (EB simplex)

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3
Q

Characteristics of blisters formed in epidermolytic EB

A

Intraepidermal vesicles with a roof of nearly intact epidermis and floor of fragmented basal keratinocytes

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4
Q

Group of autosomal recessive EB which form blisters within lamina lucida. Healing occurs without scarring, but may include residual atrophy

A

Junctional EB

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5
Q

Type of junctional EB associated with mutations in genes for laminin and integrin isoforms

A

Severe junctional EB

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6
Q

Type of junctional EB associated with mutations in the gene for type XVII collagen

A

Benign junctional EB

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7
Q

Characteristics of blisters formed in junctional EB

A

Intraepidermal vesicles with a roof of intact epidermis and floor of intact lamina densa

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8
Q

Group of autosomal recessive and autosomal dominant EB attributed to defects in anchoring fibrils (type VII collagen) where blisters are deep to the lamina densa and healing involves atrophic scarring

A

Dermolytic (dystrophic) EB

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9
Q

Characteristics of blisters formed in dermolytic (dystrophic) EB

A

Subepidermal vesciles with a roof of normal epidermis an intact lamina lucida and lamina densa and floor of outer papillary dermis

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10
Q

Type of autosomal recessive EB attributed to mutation in FERMT1 gene encoding for the adhesion protein, kindling-1

A

Kindler syndrome

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11
Q

Kindler syndrome is set apart from other inherited EBs by clinical presentation of

A

Mottled skin pigmentation (poikiloderma) and photosensitivity

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12
Q

Subepidermal, autoimmune blistering disease where complement-fixing IgG targets basement membrane proteins, BPAG1 and BPAG2

A

Bullous Pemphigoid (BP)

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13
Q

Complement proteins which promote degranulation of mast cells in Bullous Pemphigoid

A

Anaphylatoxins C3a and C5a

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14
Q

Ultimate cause of disruption of anchoring filaments, breakdown of lamina densa, and vesicle formation at the lamina lucida following release of chemotactic factors (IL-5 & Eotaxin) by mast cells

A

Eosinophilic degranulation at the dermal-epidermal junction

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15
Q

Characteristics of blisters formed in Bullos Pemphigoid

A

Subepidermal blister with a roof of intact epidermis and floor of lamina densa

Blister contains eosinophils, lymphocytes, neutrophils, and fibrin

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16
Q

Treatment typically used to shorten course of Bullous Pemphigoid

A

Systemic administration of corticosteroid

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17
Q

Intensely pruritic cutaneous eruptions with small subepidermal vesicles associated with gluten sensitivity and IgA complex deposition at the dermal papillae

A

Dermatitis Herpetiformis

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18
Q

Immune cells typically seen at the tips of dermal papillae in response to IgA deposition in dermatitis herpetiformis

A

Neutrophils

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19
Q

Acute, self-limited disorder ranging from formation of erthematous macules and blisters to ulcers which are usually a reaction to a drug or infectious agent such as herpes simplex

A

Erythema multiforme

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20
Q

An unusually severe, and frequently fatal, form or erythema multifome involving mucosal surfaces and internal organs

A

Stevens-Johnson syndrome

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21
Q

Autoimmune disorder associated with epidermal injury due to exogenous agents, such as UV light, which typically presents with malar or butterfly rash, immune complexes in the basement membrane zone, and elevated antinuclear antibodies (ANA)

A

Acute systemic lupus erythematosus

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22
Q

Cell mediated immunologic reaction to epidermal injury resulting in saw-tooth appearing inflammation

A

Lichen Planus

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23
Q

In lichen planus, what characteristically appears at the dermal-epidermal junction

A

band-like congregation of lymphocytes

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24
Q

Lichen Planus is associated with which viral infection

A

Hepatitis C

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25
Q

Basal cell layer destruction and subsequent reactive epidermal proliferation in Lichen Planus leads to what characterization in each epidermal layer

A

Stratum corneum: Hyperkeratosis without parakeratosis
Stratum granulosum: thickened in an often wedge-shaped pattern
Stratum spinosum: variably thickened
Stratum basale: cuboidal keratinocytes replaced by flattened or polygonal keratinocytes

26
Q

Clinical features of Lichen Planus include

A

Violet colored (violaceious), flat-topped papules and Wickham striae

27
Q

Lichen planus typically affects

A

flexor surfaces and wrists

28
Q

Oral mucous membrane involvement in Lichen Planus manifests as

A

Wickhamm striae

29
Q

IgE-dependent hypersensitivity reaction with degranulization of mast cells involving edema of the superficial dermis

A

Urticaria (hives)

30
Q

With urticaria, within few hours of offending agent, what skin lesions appear

A

Pruritic papules and plaques

31
Q

IgE-dependent hypersensitivity reaction, with degranulization of mast cells, involving egg-like swelling from edema in the deeper dermis or subcutis

A

Angioedema

32
Q

Heritable angioedema is associated with mutation in

A

C1-esterase inhibitor

33
Q

Both Urticaria and Angioedema are treated with

A

Antihistamines

34
Q

Exaggerated IgE-dependent response which allows you to write on your skin and form hives in the shape of legible words

A

Dermatographism

35
Q

Immune reaction which presents as palpable purpura that doesn’t blanche under pressure

A

Cutaneous necrotizing vasculitis (CNV)

36
Q

CNV is typically due to neutrophil degranulation and vessel damage following

A

Immune complex deposition on vessel walls

37
Q

Cutaneous necrotizing vasculitis is also known as

A

hypersensitivity angiitis

38
Q

The cases of CNV which are not of unknown etiology or related to a chronic disease are often associated with

A

An infectious agent (ex: Hep B virus, Streptococcus)

39
Q

Three main characteristics of vascular damage in CNV are

A

Extravasation of erthryocytes
Fibrin deposition
Leukocytoclasia

40
Q

Cell-mediated hypersensitivity to allergens which involves epidermal edema and a pruritic, erythematous, exudative rash

A

Allergic contact dermatitis

41
Q

Allergic contact dermatitis is a model of

A

Spongiotic dermatitis

42
Q

Which phase of allergic contact dermatitis involves migration of Langerhans cells into dermal lymphatics and subsequent presentation to CD4+ T cells

A

Sensitization phase

43
Q

Which phase of allergic contact dermatitis involves epidermal entry of sensitized CD4 T cells and release of cytokines after re-exposure to allergen

A

Elicitation phase

44
Q

Three common allergens for contact dermatitis include

A

Hapten containing plants (ex: poison ivy)
Irritant chemicals (ex: detergents)
Drugs (ex: penicillin)

45
Q

General term referring to cell-mediated immune response to indigestible antigens in the epithelium

A

<p>Granulomatous dermatitis </p>

46
Q

Multiorgan systemic disorder of unknown etiology whose skin lesions present as noncaseating granulomas in the reticular dermis

A

Sarcoidosi

47
Q

Benign, self-limited disorder characterized by necrobiosis, or focal collagen degeneration, surrounded by palisaded macrophages

A

Granuloma annulare

48
Q

Areas typically affected by granuloma annulare

A

Dorsum of the hands and feet

49
Q

Disorder of the reticular dermal connective tissue marked by fibrosis and tightening of the skin

A

Scleroderma

50
Q

Important clinical feature of early scleroderm

A

Raynaud phenonmenon or non pitting edema of hands or fingers

51
Q

Involvement of the esophagus in scleroderma can lead to

A

Dysphagia

52
Q

Disorder similar to sceleroderma, but only involving patchy, circumscribed areas of skin

A

Morphea

53
Q

Heterogenous group of diseases characterized by inflammation of subcutaneous tissue

A

Panniculitis

54
Q

Cutaneous disorder with self-limited, nonsuppurative, tender nodules over extensor surfaces of lower extremities

A

Erythema nodusum

55
Q

Histologically, erythema nodosum is characterized by

A

Necrosis and fibrosis of adipose tissue
Neutrophilic inflammation
Foreign body giant cells
Inflammatory cells

56
Q

Disorder of chronic, recurrent subcutaneous nodules of plaques on the legs associated with mycobacterium tuberculosis

A

Erythema induratum

57
Q

Self-limited inflammatory disorder of sebaceous follicles resulting in papular or pustrular lesions

A

Acne vulgaris

58
Q

Excess keratin production at the neck of follicles and hormone associated increases in sebum production forms

A

Comedones (plugged follicles)

59
Q

What are the two types of comedones

A

Blackheads (open comedones)

Whiteheads (closed comedones)

60
Q

Anaerobic diphtheroid associated with acne vulgaris which releases hydrolytic enzymes and forms pustules or follicular abscesses

A

Propionibacterium acnes

Pathology Unit 2 (17 decks)

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