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Dermatology > Bullous Disorders > Flashcards

Bullous Disorders Flashcards

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Dermatology Board Prep flashcards
1
Q

In pemphigus valgaris is the level of blistering intra-epidermal or sub-epidermal?

A

Intra-epidermal (s=superficial)

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2
Q

In bullous pemphigoid is the level of blistering intra-epidermal or sub-epidermal?

A

Sub-epidermal (d=deep)

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3
Q

What could the investigations be for blistering caused by infection?

A

Bacterial microscopy & culture
PCR - HSV, VZV
Viral culture
Serology

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4
Q

What would the investigation be for blistering caused by porphyria?

A

Porphyrin studies

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5
Q

What would the investigation be for blistering caused by contact dermatitis?

A

Patch testing

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6
Q

What would the investigations be for blistering caused by an autoimmune disorder?

A

Biopsy with IMF (immunofluorescence)

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7
Q

Name 3 autoimmune bullous diseases?

A

Bullous pemphigoid
Pemphigus vulgaris
Dermatitis herpetiformis (DH)

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8
Q

What is Nikolsky sign?

A

Slight rubbing of the skin results in exfoliation of the outermost layer (Nikolsky sign positive)

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9
Q

Describe bullous pemphigoid?

A

Large tense bullae on normal skin or erythematous base

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10
Q

Is bullous pemphigoid Nikolsky sign positive or negative?

A

Negative

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11
Q

Describe the pathogenesis of bullous pemphigoid?

A

Circulating antibodies (IgG) react with antigens in the basement membrane (BM) and hemidesmosomes anchoring basal cells to BM

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12
Q

What would a biopsy with IMF show in bullous pemphigoid?

A

Linear IgG and complement deposited around the BM

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13
Q

What is the treatment for localised bullous pemphigoid?

A

Topical potent steroids

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14
Q

What is the treatment for generalised bullous pemphigoid?

A

Oral prednisolone (40-80mg/day) combined with an oral immunosuppressant (e.g. azathioprine, ciclosporin) or tetracycline

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15
Q

Describe pemphigus vulgaris?

A

Flaccid vesicles/bullae on scalp, face, axillae, groins

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16
Q

Is pemphigus vularis Nikolsky sign positive or negative?

A

Positive

17
Q

Describe the pathogenesis of pemphigus vulgaris?

A

IgG antibodies are directed against intercellular adhesions

18
Q

What would a biopsy with IMF show in pemphigus vulgaris?

A

Cell surface bound IgG, chicken wire pattern in epidermis

19
Q

What is acantholysis? (a process common to all variants of pemphigus)

A

Acantholysis is the loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes

20
Q

What is the treatment for local pemphigus vulgaris?

A

Topical steroids

Topical anaesthetics

21
Q

What is the treatment for systemic pemphigus vulgaris?

A

Prednisolone +/- azathioprine

22
Q

Dermatitis herpetiformis is associated with what other autoimmune disease?

A

Coeliac disease

23
Q

Describe dermatitis herpetiformis?

A

Small blisters on erythematous urticarial base with itch

24
Q

What is the pathophysiology of dermatitis herpetiformis?

A

IgA autoantibodies to gluten tissue transaminase in the gut and epidermis

25
Q

In dermatitis herpetiformis is the level of blistering intra-epidermal or sub-epidermal?

A

Sub-epidermal

26
Q

What are the investigations for dermatitis herpetiformis?

A
  1. Coeliac serology - IgA antibodies to tissue transglutaminase (tTG)
  2. Histology
  3. Small intestinal scope + biopsy - partial or subtotal villous atrophy
27
Q

What would a biopsy with IMF show in dermatitis herpetiformis?

A

Granular IgA in dermal papillary tips

28
Q

What is the treatment for dermatitis herpetiformis?

A

Gluten-free diet + Dapsone

29
Q

What needs to be checked before a patient is put on dapsone and why?

A

Blood glucose-6-phosphate dehydrogenase levels as a deficiency increases the risk of haemolysis due to dapsone

30
Q

What is a recognised complication of dermatitis herpetiformis?

A

Small bowel lymphoma

Dermatology (16 decks)

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