Vesiculobullous blistering Flashcards

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1
Q

what is the primary feature of immunobullous disorders

A

blisters

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2
Q

what is nikosky’s sign

A

the top layers of the skin slip away from each other when rubbed gently

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3
Q

what is nikosky’s sign postive

A

pemphigus vulgaris

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4
Q

pemphigus vulgaris

A

produces fragile fluid filled blisters that rupture to form shallow erosions

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5
Q

where is the split in pemphigus vulgaris

A

superficial and intraepidermal

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6
Q

where does pemphigus vulgaris typically effect

A

scalp, face, axillae and groin and oral area.

mucosal involvement eg eyes, genitals common

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7
Q

what is there a risk of when lesions rupture

A

infection 2y

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8
Q

pemphigus vulgaris pathpphysiology

A
  • IgG antibodies made against desmoglein 3, immune complexes form and results in loss of intercellular connections (desmosomes). This results in loss of cohesion between keratinocytes – ACANTHOLYSIS
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9
Q

where is desmoglein 3 found

A

found in keratinocytes

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10
Q

Bullous Pemphigoid usually presents in

A

elderly , background of dermatitis or normal skin

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11
Q

Bullous Pemphigoid

A

dense blisters and erosions

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12
Q

where is the split in Bullous Pemphigoid

A

through DEJ

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13
Q

do Bullous Pemphigoid heal with/out scarring

A

without scarring

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14
Q

what is the prebullous period in Bullous Pemphigoid

A

can be prolonged - persistent pruritic urticated plaques or eczema precedes blisters

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15
Q

does mucous involvement occur in Bullous Pemphigoid

A

in about 20% cases

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16
Q

does acantholysis occur in Bullous Pemphigoid or pemphigus vulgaris

A

pemphigus vulgaris

17
Q

Bullous Pemphigoid pathophysiology

A

Hemidesmosomes are found in the basement membrane zone, and act to keep the epidermis attached to the dermis. Circulating antibodies (IgG) attack the hemidesmosomes in the BM, causing the epidermis to float off from BM.

18
Q

what are hemidesmosomes

A

The hemidesmosomes are found in the BM zone of epidmermis of skin and act to keep the epidermis attached to the dermis normally

19
Q

what drugs can induce Bullous Pemphigoid

A

ACE inhibitors, penicillin and furosemide

20
Q

Bullous Pemphigoid prognosis

A

chronic self limiting course . most patients achieve remission on treatment within 3-6 months

21
Q

what is the mortality of pemphigus like if left untreated

A

high

22
Q

investigations

A

skin biopsy with direct immunofluorescence and indirect immunofluorescence

23
Q

Dermatitis Herpetiformis - genes

A

HLA DQ2 and DQ8

24
Q

how is PV treated

A

PV: 60-100m daily oral prednisolone. IS agents are used as steroid sparing agents.

25
Q

how is BP treated

A
  • oral or systemic steroids
  • antibiotics for 2y bacterial infection
  • Mild disease can be treated with high dose oral prednisolone (30-60mg daily) and steroid-sparing agents
  • tetracyclines (doxycycline) can be used as a steroid sparing agent