Ch 24: Skin: BM, Vascular, Acne

Question 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

Answer 1

epidermolysis bullosa (EB)

Question 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

Answer 2

Epidermolytic EB (EB simplex)

Question 3

Characteristics of blisters formed in epidermolytic EB

Answer 3

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

Question 4

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

Answer 4

Junctional EB

Question 5

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

Answer 5

Severe junctional EB

Question 6

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

Answer 6

Benign junctional EB

Question 7

Characteristics of blisters formed in junctional EB

Answer 7

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

Question 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

Answer 8

Dermolytic (dystrophic) EB

Question 9

Characteristics of blisters formed in dermolytic (dystrophic) EB

Answer 9

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

Question 10

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

Answer 10

Kindler syndrome

Question 11

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

Answer 11

Mottled skin pigmentation (poikiloderma) and photosensitivity

Question 12

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

Answer 12

Bullous Pemphigoid (BP)

Question 13

Complement proteins which promote degranulation of mast cells in Bullous Pemphigoid

Answer 13

Anaphylatoxins C3a and C5a

Question 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

Answer 14

Eosinophilic degranulation at the dermal-epidermal junction

Question 15

Characteristics of blisters formed in Bullos Pemphigoid

Answer 15

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

Blister contains eosinophils, lymphocytes, neutrophils, and fibrin

Question 16

Treatment typically used to shorten course of Bullous Pemphigoid

Answer 16

Systemic administration of corticosteroid

Question 17

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

Answer 17

Dermatitis Herpetiformis

Question 18

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

Answer 18

Neutrophils

Question 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

Answer 19

Erythema multiforme

Question 20

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

Answer 20

Stevens-Johnson syndrome

Question 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)

Answer 21

Acute systemic lupus erythematosus

Question 22

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

Answer 22

Lichen Planus

Question 23

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

Answer 23

band-like congregation of lymphocytes

Question 24

Lichen Planus is associated with which viral infection

Answer 24

Hepatitis C

Question 25

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

Answer 25

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

Question 26

Clinical features of Lichen Planus include

Answer 26

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

Question 27

Lichen planus typically affects

Answer 27

flexor surfaces and wrists

Question 28

Oral mucous membrane involvement in Lichen Planus manifests as

Answer 28

Wickhamm striae

Question 29

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

Answer 29

Urticaria (hives)

Question 30

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

Answer 30

Pruritic papules and plaques

Question 31

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

Answer 31

Angioedema

Question 32

Heritable angioedema is associated with mutation in

Answer 32

C1-esterase inhibitor

Question 33

Both Urticaria and Angioedema are treated with

Answer 33

Antihistamines

Question 34

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

Answer 34

Dermatographism

Question 35

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

Answer 35

Cutaneous necrotizing vasculitis (CNV)

Question 36

CNV is typically due to neutrophil degranulation and vessel damage following

Answer 36

Immune complex deposition on vessel walls

Question 37

Cutaneous necrotizing vasculitis is also known as

Answer 37

hypersensitivity angiitis

Question 38

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

Answer 38

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

Question 39

Three main characteristics of vascular damage in CNV are

Answer 39

Extravasation of erthryocytes Fibrin deposition Leukocytoclasia

Question 40

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

Answer 40

Allergic contact dermatitis

Question 41

Allergic contact dermatitis is a model of

Answer 41

Spongiotic dermatitis

Question 42

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

Answer 42

Sensitization phase

Question 43

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

Answer 43

Elicitation phase

Question 44

Three common allergens for contact dermatitis include

Answer 44

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

Question 45

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

Answer 45

Granulomatous dermatitis

Question 46

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

Answer 46

Sarcoidosi

Question 47

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

Answer 47

Granuloma annulare

Question 48

Areas typically affected by granuloma annulare

Answer 48

Dorsum of the hands and feet

Question 49

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

Answer 49

Scleroderma

Question 50

Important clinical feature of early scleroderm

Answer 50

Raynaud phenonmenon or non pitting edema of hands or fingers

Question 51

Involvement of the esophagus in scleroderma can lead to

Answer 51

Dysphagia

Question 52

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

Answer 52

Morphea

Question 53

Heterogenous group of diseases characterized by inflammation of subcutaneous tissue

Answer 53

Panniculitis

Question 54

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

Answer 54

Erythema nodusum

Question 55

Histologically, erythema nodosum is characterized by

Answer 55

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

Question 56

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

Answer 56

Erythema induratum

Question 57

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

Answer 57

Acne vulgaris

Question 58

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

Answer 58

Comedones (plugged follicles)

Question 59

What are the two types of comedones

Answer 59

Blackheads (open comedones) | Whiteheads (closed comedones)

Question 60

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

Answer 60

Propionibacterium acnes