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Year 4 - Dermatology > Bullous Disorders > Flashcards

Bullous Disorders Flashcards

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Dermatology Board Prep flashcards
1
Q

Describe the blisters in bullous pemphigoid

A

Subepidermal

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2
Q

Describe the blisters in pemphigus vulgaris

A

Intra-epidermal

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3
Q

Which is more likely to present with erosions: pemphigoid or pemhigus

A

Pemphigus

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4
Q

Which is pemphigoid more likely to present with large tense bullae?

A

The blister is under the dermis so it lifts this entire layer to make a strong stable roof

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5
Q

How should you investigate bullous disorders?

A

Depends on what you suspect is the cause:

Infection - microscopy
Porphyria - porphyrin study
Contact dermatitis - patch testing
Autoimmune - biopsy with IF

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6
Q

What is the typical presentation of bullous pemphigoid?

A 
>60yrs
Large tense bullae
Itchy erythematous plaques
No scarring
Mucosal lesions only in mouth
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7
Q

Outline the pathogenesis of pemphigoid

A

IgG autoantibodies attach BP180 and BP120 protein in hemidesmosomes
Can’t attach basal cells to the basement membrane
Lets fluid in

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8
Q

What does IF show in pemphigoid?

A

Linear IgG and complement around the basement membrane

basically a (green?) line surrounding by (amber) dots

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9
Q

How do you treat for generalised bullous pemphigoid?

A

Prednisolone 40-80mg daily for 2 weeks
Tetracyclines
Methotrexate

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10
Q

True or false:

Relapse is common with pemphigoid

A

False

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11
Q

How long does the average patient take to go into remission with pemphigoid?

A

3-6 months

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12
Q

How would you treat localised pemphigoid?

A

Highly potent topical steroids

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13
Q

Which is more common: pemphigoid or pemphigus?

A

Pemphigoid

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14
Q

Why are pemphigus blisters more likely to burst?

A

Intra-epidermal so they have a thinner roof

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15
Q

Where are skin lesions commonly found with pemphigus?

A

Scalp, face, axillae, groin

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16
Q

What is a positive Nikolsy sign?

A

When you can induce a blister in a patient but rubbing the skin
OR
you can shift a small blister sideways

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17
Q

What is the target of the autoantibodies in pemphigus?

A

Desmoglein proteins

Mostly type 3

18
Q

What is desmolgein?

A

A membrane protein in the membrane of the cells in the spinous layer

19
Q

What is the role of desmoglein?

A

Maintain integrity of the hemidesmosomes

20
Q

What clinical sign may precede bullae in pemphigoid?

A

Itchy erythematous plauqes and papules for up to 1 year before

(these will not respond to low intensity steroids of fungal creams)

21
Q

Which type of blistering occurs in dermatitis herpetiformis?

A

Subepidermal

22
Q

Is Nikolsky’s sign positive in pemphigus, pemphigoid, DH or all?

A

Pemphigus

23
Q

What does IF show in pemphigus?

A

IgG antibodies around the membrane in a “chicken wire pattern”

(the membrane (green?) looks like barbed wire and there are (amber?) dots in the gaps in the barbed wire)

24
Q

Which is harder to treat: pemphigus or pemphigoid?

A

Pemphigus

25
Q

How do you treat local pemphigus?

A

Topical steroid and anaesthetics

26
Q

How do you treat systemic pemphigus?

A

Prednisolone +/- DMARD therapy

27
Q

What is the mortality rate in pemphigus?

A

10-20%

28
Q

How long on average does it take to go into remission with pemphugus?

A

3-6 years

29
Q

Which other condition is DH strongly linked to?

A

Coeliac disease

30
Q

What is the classic presentation of pemphigus?

A

Small blisters on erosions on scalp, neck, axillae, groin and all mucosal regions

31
Q

How does DH present?

A

Itchy small blisters and erosions and excoriation on the buttocks, lower back and extensor surfaces

32
Q

Which mucosal surfaces are involved in DH?

A

Rare to have any mucosal membrane involvement

33
Q

How do you investigate DH?

A

Biopsy of uninvolved skin
Histology
IF

34
Q

What will biopsy show in DH?

A

Granular deposits of dermal papillary IgA

35
Q

What will histology show in DH?

A

Subepidermal blisters
Micro abscesses in dermal papillae
Basal laminae lifted off

36
Q

Which cancer is assocaited with DH?

A

Lymphoma but very rare

37
Q

Which treatment should give fast relief of itch?

A

Dapsone

38
Q

What may small intestine biopsy show in DH?

A

Partial or subtotal villous atrophy in 2/3rds of patients

39
Q

What is the target of the antibodies in DH?

A

Transglutaminase 3

40
Q

Where is TG3 normally present?

A

In the granular layer

41
Q

How is DH treated?

A

Gluten free diet
Dapsone

(response to dapsone so good within 48 hrs that if it doesn’t help reconsider ur diagnosis)

Year 4 - Dermatology (7 decks)

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