Ch 24: Skin: BM, Vascular, Acne Flashcards Preview

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

Flashcards in Ch 24: Skin: BM, Vascular, Acne Deck (60):
1

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

epidermolysis bullosa (EB)

2

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

Epidermolytic EB (EB simplex)

3

Characteristics of blisters formed in epidermolytic EB

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

4

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

Junctional EB

5

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

Severe junctional EB

6

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

Benign junctional EB

7

Characteristics of blisters formed in junctional EB

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

8

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

Dermolytic (dystrophic) EB

9

Characteristics of blisters formed in dermolytic (dystrophic) EB

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

10

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

Kindler syndrome

11

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

Mottled skin pigmentation (poikiloderma) and photosensitivity

12

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

Bullous Pemphigoid (BP)

13

Complement proteins which promote degranulation of mast cells in Bullous Pemphigoid

Anaphylatoxins C3a and C5a

14

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

Eosinophilic degranulation at the dermal-epidermal junction

15

Characteristics of blisters formed in Bullos Pemphigoid

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

Blister contains eosinophils, lymphocytes, neutrophils, and fibrin

16

Treatment typically used to shorten course of Bullous Pemphigoid

Systemic administration of corticosteroid

17

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

Dermatitis Herpetiformis

18

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

Neutrophils

19

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

Erythema multiforme

20

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

Stevens-Johnson syndrome

21

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)

Acute systemic lupus erythematosus

22

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

Lichen Planus

23

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

band-like congregation of lymphocytes

24

Lichen Planus is associated with which viral infection

Hepatitis C

25

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

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

Clinical features of Lichen Planus include

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

27

Lichen planus typically affects

flexor surfaces and wrists

28

Oral mucous membrane involvement in Lichen Planus manifests as

Wickhamm striae

29

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

Urticaria (hives)

30

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

Pruritic papules and plaques

31

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

Angioedema

32

Heritable angioedema is associated with mutation in

C1-esterase inhibitor

33

Both Urticaria and Angioedema are treated with

Antihistamines

34

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

Dermatographism

35

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

Cutaneous necrotizing vasculitis (CNV)

36

CNV is typically due to neutrophil degranulation and vessel damage following

Immune complex deposition on vessel walls

37

Cutaneous necrotizing vasculitis is also known as

hypersensitivity angiitis

38

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

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

39

Three main characteristics of vascular damage in CNV are

Extravasation of erthryocytes
Fibrin deposition
Leukocytoclasia

40

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

Allergic contact dermatitis

41

Allergic contact dermatitis is a model of

Spongiotic dermatitis

42

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

Sensitization phase

43

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

Elicitation phase

44

Three common allergens for contact dermatitis include

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

45

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

Granulomatous dermatitis

46

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

Sarcoidosi

47

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

Granuloma annulare

48

Areas typically affected by granuloma annulare

Dorsum of the hands and feet

49

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

Scleroderma

50

Important clinical feature of early scleroderm

Raynaud phenonmenon or non pitting edema of hands or fingers

51

Involvement of the esophagus in scleroderma can lead to

Dysphagia

52

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

Morphea

53

Heterogenous group of diseases characterized by inflammation of subcutaneous tissue

Panniculitis

54

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

Erythema nodusum

55

Histologically, erythema nodosum is characterized by

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

56

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

Erythema induratum

57

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

Acne vulgaris

58

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

Comedones (plugged follicles)

59

What are the two types of comedones

Blackheads (open comedones)
Whiteheads (closed comedones)

60

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

Propionibacterium acnes