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19 Skin Pathology > 1.2 Blistering dermatoses > Flashcards

1.2 Blistering dermatoses Flashcards

1
Q

dermatitis herpetiformis

  • mech
  • associated with what
A
  • autoimmune IgA deposition on tips of dermal papillae
  • form pruritic vesicles and bullae that are grouped (look like herpes)
  • strong association with Celiac disease. treating Celiac (gluten free) will also treat dermatitis herpetiformis
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1
Q

mucous membrane pemphigoid (MMP)

A
  • blistering of mucous membranes and sometimes skin’
  • possible blindness
  • majority of pts have Ab against BP 180, but some have Ab to Bp 230 or laminin 332
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2
Q

what is it?

  • skin bullae. oral mucosa spared.
  • bullae do not rupture easily
A

bullous pemphigoid

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2
Q

what can erythema multiforme progress to?

A
  • Stevens-Johnson syndrome (SJS) if oral mucosa/lip and fever involved
  • toxic epidermal necrolysis (TEN) is severe SJS
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2
Q

bullous pemphigoid has Ab against which 2 things?

A

Hemidesmosomes:

  1. BP 230–plakin protein, important for cytoskeleton architecture
  2. BP 180–Collagen type 17
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3
Q

how does SJS/TEN typically present?

A
  • painful red macules on body, plus oral mucosa ulcers. rapidly evolves to bullae and necrosis
  • preceded by Prodome of 1-14 days, flu-like symptoms
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4
Q

what is it?

  • skin and oral mucosa bullae
  • bullae rupture easily
A

pemphigus vulgaris

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6
Q

bullous pemphigoid

  • clinical findings
  • histology
A
  • skin bullae. oral mucosa spared.
  • bullae do not rupture easily (epidermal layer above blister is thick)
  • histology: blister between epidermis and dermis. no ‘tombstone’ appearance.
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7
Q

Tx for pemphigus vulgaris

A
  • autoimmune, so:
  • prednisone
  • azathioprine (6-MP)
  • mycophenilate mofetil
  • rituximab
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8
Q

Bullous pemphigoid

-mech

A
  • autoimmune destruction of hemidesmosomes, between basal cells and dermis (basement membrane)
  • IgG against hemidesmosomes
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8
Q

main components of basement membrane (4)

A
  1. hemidesmosomes
  2. lamina lucida
  3. lamina densa
  4. sublamina densa
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9
Q

ichthyosis vulgaris

  • clinical findings
  • associated with what
A
  • ‘fish scales’ especially on shins
  • dry skin
  • associated with allergies, asthma
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11
Q

pemphigus vulgaris

-mech

A
  • autoimmune destruction of desmosomes
  • IgG against desmoglein (type 2 HSR)
  • desmosome exist in stratum spinosum of epidermis, so blister forms in that layer
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12
Q

what is it?

-pruritic vesicles and bullae that are grouped, looks like herpes

A

dermatitis herpetiformis

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13
Q

erythema multiforme

-what causes it?

A
  • cutaneous hypersensitivity reaction
    1. viral infections most common:
  • HSV infection 50% (also mycoplasma)
    2. Drug reaction
  • Abx, NSAIDs, sulfa drugs, antiepileptics
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14
Q

Epidermolysis Bullosa Acquisita (EBA)

A
  • mutation in collagen 7 in sublamina densa
  • rare autoimmune blistering disease
16
Q

erythema multiforme

  • mech
  • clinical findings
A

“multiple erythematous rashes”

  • hypersensitivity reaction
  • “targetoid” rash–looks like targets because center is necrosed
18
Q

pemphigus vulgaris

  • clinical findings
  • histology
A
  • skin and oral mucosa bullae (blisters)
  • blisters rupture easily (because epidermis superficial to blister is thin)
  • histology: ‘tombstone’ appearance of stratum basalis
19
Q

toxic epidermal necrolysis

A

severe form of stevens-johnson syndrome, which is erythema multiforme with lip/oral mucosa involvement, plus fever.

-usu drug reaction

19
Q

what is in the lamina densa?

A
  • collagen 4 and laminins
  • most important laminin is laminin 332
21
Q

what is Direct IF microscopy?

A
  • tests for IgG and C3 bound to antigens in sample of patient’s skin cells
  • test for pemphigus, etc
22
Q

generalized atrophic benign epidermolysis bullosa (GABEB)

A
  • mutated BP 180 protein (collagen 17)
  • results in blistering skin
23
Q

Nikolsky’s sign

A
  • blisters easily expanded and erupted by rubbing.
  • found in pemphigus vulgaris
24
Q

erythema multiforme

-tx?

A

-topical corticosteroids

25
Q

what is Indirect IF microscopy?

A
  • test for IgG in patient’s serum.
  • react patient’s serum with a test tissue (eg monkey tissue) to see if reaction occurs
  • test for pemphigus, etc
26
Q

Ichthyosis vulgaris

-mech

A
  • mutation in profilaggrin gene
  • profilaggrin codes for filaggrin protein
  • auto dom, 1/250 so common
27
Q

Herlitz junctional epidermolysis bullosa

A
  • congenital mutation in laminin 332 in lamina densa
  • blisters, lethal
28
Q

In erythema multiformes cause by herpes, why is it not helpful to tx with acyclovir?

A
  • Too late:
  • rash usually appears after HSV lesions have resolved.
29
Q

stevens-johnson syndrome

A

erythema multiforme, with oral mucosa/lip involvement and fever

19 Skin Pathology (8 decks)

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