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FCM > Block 1- Bullous disorders > Flashcards

Block 1- Bullous disorders Flashcards

1
Q

Vesicles are ___ blister

A

small

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2
Q

bull are ___ blister

A

LARGE

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3
Q

name disease:

A potentially life-threatening autoimmune mucocutaneous disease - mortality rate of approximately 5-15%

A

Pemphigus vulgaris

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4
Q

before ______, Pemphigus vulgaris

was fatal

A

glucocorticoids

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5
Q

name disease:

  • intraepidermal, blistering disease
  • affects skin and mucous membranes
  • mediated by circulating autoantibodies directed autoantibodies against the connections between epidermal cells
A

Pemphigus vulgaris

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6
Q

intrapidermal blister is associated with PV or BP?

A

Pemphigus vulgaris

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7
Q

Pemphigus vulgaris is mediated by circulating autoantibodies directed autoantibodies against the connections between ____ cells

A

epidermal

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8
Q

Epidemiology of Pemphigus vulgaris

A

Age: Younger patient- <60 yrs (vs. Bullous pemphigoid >60 yrs)
Sex: Male=Female

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9
Q

Pemphigus vulgaris CLINICAL COURSE

A

First: Painful oral erosions occur 1st (NOT Pruritic)

Next: skin blisters – may be delayed weeks or months

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10
Q

do they bullae of Pemphigus vulgaris easily rupture?

A

yes

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11
Q

does Pemphigus vulgaris have a positive or negative Nikolsy’s sign?

A

positive

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12
Q

what does it mean when there is a positive Nikolsy’s sign in Pemphigus vulgaris?

A

Application of pressure to small intact bullae causes the fluid to dissect laterally into the midepidermal areas

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13
Q

most common sites where your find Pemphigus vulgaris

A

o Oral cavity (other mucosal sites later in course)
o scalp
o face
o axillae

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14
Q

how to diagnose Pemphigus vulgaris?

A
  • Skin Biopsy [Bx]

- Immunofluorescence of PERILESIONAL skin [in Michel’s medium]

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15
Q

treatment for Pemphigus vulgaris?

A

CORTICOSTEROIDS AND IMMUNOSUPPRESSIVE AGENTS

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16
Q

is remission of Pemphigus vulgaris possible?

A

yes

17
Q

in about 75% of pts, you can discontinue treatment of Pemphigus vulgaris in
__ years.

A

10

18
Q

Differential Diagnosis for pemphigus vulgaris

A
  • bullous pemphigoid (think elderly)
  • epidermolysis bullosa
  • dermatitis herpetiformis [gluten]
  • bullous systemic lupus erythematosus
  • bullous drug eruptions
19
Q

prognosis for pemphigus vulgaris

A
  • Tripled mortality rate vs unaffected population
  • Complications secondary to the use of high-dose corticosteroids contribute to the mortality rate
  • Morbidity and mortality are related to the extent of disease, the maximum dose of systemic steroids required to induce remission, and the presence of other diseases
  • Prognosis worse with extensive pemphigus vulgaris and in older aged patients

FCM (9 decks)

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