DERM - Vesicular lesions of the skin Flashcards

1
Q

top to bottom, what are the layers of the skin?

A
  • corneum
  • granulosum
  • spinosum
  • basal
  • basement membrane
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2
Q

the basement membrane is held onto the epidermis by what proteins?

A
  • BPAG1

- BPAG2

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3
Q

loss of what proteins leads to a subepidermal blister?

A
  • BPAG1

- BPAG2

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4
Q

loss of BPAG1 and BPAG2 lead to what type of lesion?

A

subepidermal blister

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5
Q

blisters can be in what layers?

A
  • subcorneal
  • intraepidermal
  • subepidermal
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6
Q

bullous pemphigoid consists of blisters in what layer?

A

subepidermal

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7
Q

what are the cell poor blisters?

A
  • porphyria cutanea tarda
  • electrical / thermal burns
  • toxic epidermal necrolysis
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8
Q

what are the blisters with inflammation?

A
  • bullous pemphigoid
  • dermatitis herpetiformis
  • linear IgA dermatosis
  • bullous lupus erythematosus
  • bullous drug eruption
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9
Q

friction blisters occur in what layer?

A

suprabasal

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10
Q

pemphigus vulgaris and vegetans occur in what layer?

A

suprabasal

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11
Q

herpes simplex and zoster blisters occur in what layer?

A

suprabasal

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12
Q

SSSS occurs in what layer?

A

subcorneal

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13
Q

impetigo occurs in what layer?

A

subcorneal

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14
Q

pemphigus foliaceous occurs in what layer?

A

subcorneal

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15
Q

direct IF consists of Abs against patient’s _______________ skin

A

perilesional

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16
Q

what is the pathogenesis of bullous pemphigoid?**

A

autoantibody against bullous pemphigoid antigens BP180 and BP230 (attach dermis to epidermis)

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17
Q

bullous pemphigoid is a disorder exclusively of the _________

A

elderly (60-80 and over)

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18
Q

what is the clinical picture for bullous pemphigoid?

A
  • prodromal eruption (urticarial / papular lesions)
  • evolves over weeks / months to a generalized bullous eruption
  • moderate to severe pruritis
  • tense bullae arise in normal or erythematous skin**
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19
Q

where is bullous pemphigoid NOT found?

A

mucosal surfaces

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20
Q

what is the term for edema between squamous cells?

A

spongiosus

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21
Q

subepidermal blister with eosinophils - diagnosis**

A

bullous pemphigoid

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22
Q

what is the IF for bullous pemphigoid?

A

linear deposition of IgG and C3 along BM

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23
Q

linear deposition of IgG and C3 along BM - diagnosis

A

bullous pemphigoid

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24
Q

what is the treatment for BP?

A

SYSTEMIC steroids (not topical) or other immunosuppressants

25
Q

what is cicatricial pemphigoid? how is it treated?

A
  • disease of elderly
  • affects mucosal surfaces
  • chronic and recurring
  • scarring
  • immunosuppressives
26
Q

intensely pruritis dermatosis of pregnancy?

A

pemphigoid gestationis

27
Q

does pruritis dermatosis of pregnancy spare the umbilicus, or involve it?

A

involves it

28
Q

what are the features of dematitis herpetiformis?

A
  • papules, vesicles, occasional bullae on erythematous or often urticarial base
  • extensor involvement (elbows and knees)
  • associated with celiac disease
29
Q

a patient with celiac disease presents with papules / vesicles on an erythematous / urticarial base on extensor surfaces - diagnosis?**

A

dermatitis herpetiformis

30
Q

what is the pathogenesis of dermatitis herpetiformis?

A

antibodies to tissue transglutaminase (TTG): deposit in skin to activate complement with subsequent chemotaxis of neutrophils

31
Q

what serum marker is used to monitor dermatitis herpetiformis?

A

TTG

32
Q

what is seen on IF in DH?

A

granular IgA deposition in dermal papillae** (clusters)

33
Q

granular IgA deposition in dermal papillae - diagnosis?

A

DH

34
Q

grouped vesicles in “cluster of jewels” appearance - diagnosis?

A

linear IgA bullous dermatosis (LABD)

35
Q

granular IgA deposition along BM** - diagnosis?

A

LABD

36
Q

PCT is due to decreased activity of what enzyme?

A
  • uroporphyrinogen decarboxylase
37
Q

what builds up in PCT?

A
  • uroporphyrins

- coproporphyrins

38
Q

sporadic PCT is associated with what disease?

A

Hep C

39
Q
  • blisters on light exposed skin particularly hands
  • tense bullae on normal appearing background

diagnosis?

A

PCT

40
Q

what is the histology of PCT?

A

cell poor subepidermal blister (non-inflammatory)

41
Q

histology shows cell poor subepidermal blister - diagnosis?

A

PCT

42
Q

what is the IF in PCT?

A

superficial blood vessels with circumferential IgG deposition

43
Q

what is the pathogenesis of pemphigus vulgaris?**

A

formation of autoantibodies to the intercellular layers of the epidermis, leading to spontaneous blistering or fissures resulting from minor trauma

44
Q

PV is more common in what demographic?

A

Jewish / mediterranean descent age 40-60

45
Q

how does PV differ from BP?

A
  • PV: numerous stages of blisters exists simultaneously
  • PV: oral mucosa involved
  • PV: can be fatal
46
Q

antibodies in PV are directed against which proteins? what are their functions?

A

desmoglein 1 and 3 (form intercellular bridges between squamous cells)

47
Q

where does PV usually start?

A

oral mucosa

48
Q

what is the gross appearance of PV?

A

flaccid vesicles on normal skin that often erode and crust from easy rupturing

49
Q

what is the IF appearance of PV?**

A

“chicken wire” of IgG or C3

50
Q

IF: “chicken wire” of IgG or C3**

A

PV

51
Q

impetigo is caused by what organisms?

A
  • staph aureus

- strep pyogenes

52
Q

the toxin responsible for SSSS and impetigo targets what protein? what is its function?

A
  • desmoglein 1

- cell to cell adhesion within uppermost epidermal layers

53
Q

what is the inheritance of hailey hailey disease?

A

AD

54
Q

hailey hailey disease involves a defect in _______ which leads to __________

A
  • intercellular stores of calcium

- decreased cellular adhesion

55
Q

where is hailey hailey disease seen?

A

felxural and intertriginous areas - axilla, groin, neck

56
Q

what is the treatment for hailey hailey disease?

A

tetracycline

57
Q

multiple discrete scaling papules in seborrheic distribution - diagnosis? what is the inheritance?

A
  • darier’s disease

- AD

58
Q

darier’s disease is due to a defect in _________ which leads to __________

A
  • intercellular stores of calcium

- decreased cellular adhesion

59
Q

presentation:

  • abrupt onset pruritic crops of lesions on trunk and proximal extremities
  • reddish small papules with vesicles and erosions
A

grover’s disease