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Clinical lectures: Yr2 > Immunobullous disease: Bullous pemphigoid, dermatitis herpetiformis, pemphigus > Flashcards

Immunobullous disease: Bullous pemphigoid, dermatitis herpetiformis, pemphigus Flashcards

1
Q

What is immunobullous disease?

A

A group of heterogeneous autoimmune diseases characterised by large cutaneous blistering caused by autoantibodies targeting proteins in the epidermis, basement membrane, and dermis.

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2
Q

What is Bullous pemphigoid?

A

A chronic autoimmune cutaneous blistering disease characterised by subepidermal bullae.

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3
Q

What is the typical age of onset for Bullous pemphigoid?

A

70 years old and above.

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4
Q

Is Bullous pemphigoid equally common in men and women?

A

True.

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5
Q

Is Bullous pemphigoid rare in children?

A

True.

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6
Q

What increases the likelihood of drug-induced Bullous pemphigoid?

A

Younger patients.

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7
Q

What are the circulating autoantibodies in Bullous pemphigoid primarily targeting?

A

Collagen XVII (BP antigen 2 BPAG2) and BPAG1.

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8
Q

Where are the antigens targeted by autoantibodies found, in bullous pemphigoid?

A

On hemidesmosomes, which provide adhesion between epidermis and dermis.

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9
Q

Name a type of drug that can induce Bullous pemphigoid.

A

Sulfur-containing drugs like furosemide, sulfasalazine, and antibiotics like amoxicillin and penicillin.

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10
Q

What are the two phases of Bullous pemphigoid?

A
  • Nonbullous phase
  • Bullous phase
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11
Q

Describe the nonbullous phase of Bullous pemphigoid.

A

Urticarial and erythematous patches with pruritus.

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12
Q

Describe the bullous phase of Bullous pemphigoid.

A

Multiple fluid-filled or blood-filled, tense bullae that become flaccid after rupture.

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13
Q

What type of bases do bullae usually have?

A

Erythematous, urticarial bases.

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14
Q

What can bullae heal without, but may leave behind?

A

Scarring but can leave postinflammatory hyperpigmentation or milia/milk spots.

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15
Q

Where are bullae typically distributed in bullous pemphigoid?

A

On flexural surfaces on extremities and trunk, usually not on head and neck.

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16
Q

Can Bullous pemphigoid involve mucosal membranes?

A

Yes, eg. oral mucosa with sparing of the lips.

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17
Q

What is the Asboe Hansen sign?

A

Extension of a large blister to adjacent non-blistered skin occurs when pressure is applied.

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18
Q

What is the Nikolsky sign?

A

Rubbing skin causes the upper epidermis to separate from the lower epidermis.

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19
Q

What is the gold standard for investigating bullous pemphigoid?

A

Direct Immunofluorescence (DIF) microscopy from perilesional skin biopsy, showing linear IgG and/or linear C3 deposits along the dermoepidermal junction.

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20
Q

What assay identifies anti-BPAG2 and anti-BPAG1 antibodies?

A

ELISA assay.

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21
Q

What does tissue biopsy histology reveal in bullous pemphigoid?

A

Subepidermal bullae with eosinophilic infiltrate.

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22
Q

What is dermatitis herpetiformis?

A

A chronic autoimmune cutaneous blistering disease strongly associated with coeliac disease.

23
Q

What is the typical epidemiology of dermatitis herpetiformis?

A

Almost all cases occur with coeliac disease or other gluten-sensitive enteropathy, but can occur without CD.

24
Q

What is the gender prevalence in dermatitis herpetiformis?

A

Overall more common in men, but more common in female children than male children.

25
Q

What is the typical age of onset for dermatitis herpetiformis?

A

In the 40s, but can occur at any age.

26
Q

Which ethnic groups are more commonly affected by dermatitis herpetiformis?

A

More common in Caucasians or North Europeans.

27
Q

What autoimmune conditions are commonly associated with dermatitis herpetiformis?

A

Common: Hashimoto’s thyroiditis, insulin-dependent diabetes.

28
Q

What autoimmune conditions are less commonly associated with dermatitis herpetiformis?

A

Uncommon: Pernicious anaemia, alopecia areata, SLE, sarcoidosis.

29
Q

What genetic predisposition is involved in dermatitis herpetiformis?

A

Specific HLA genes involved in processing gliadin antigen.

30
Q

What is the pathophysiology of dermatitis herpetiformis?

A

Genetic predisposition causes IgA autoantibodies to form in response to gliadin and epidermal transglutaminase expressed by keratinocytes, as well as tissue transglutaminase associated with coeliac.

31
Q

What is the clinical presentation of dermatitis herpetiformis?

A

Bilateral and symmetrical, grouped vesicles and papules that are extremely pruritic.

32
Q

Where are the lesions of dermatitis herpetiformis typically distributed?

A

On extensor surfaces, upper back, buttocks, and scalp.

33
Q

What do patients usually present with in dermatitis herpetiformis?

A

Pinpoint erosions and excoriations due to pruritus, with rarely intact vesicles.

34
Q

What is the gold standard for investigating pemphigus?

A

Direct Immunofluorescence microscopy from perilesional skin biopsy shows discontinuous, granular deposits of IgA selectively localised in the tips of dermal papillae.

35
Q

What assay identifies epidermal transglutaminase (eTG) IgA autoantibodies?

A

ELISA assay.

36
Q

What test is used for coeliac disease?

A

TTG test.

37
Q

What does tissue biopsy histology show in dermatitis herpetiformis?

A

Subepidermal bullae with neutrophilic infiltrate.

38
Q

What characterizes pemphigus?

A

Group of chronic autoimmune blistering disorders with blisters on the skin and mucosal membranes.

39
Q

What do circulating IgG autoantibodies target in pemphigus?

A

Desmoglein 3 (Dsg 3) and desmoglein 1 (Dsg 1), which are antigens on desmosomes.

40
Q

What type of split is seen in pemphigus foliaceous?

A

Subcomeal split.

41
Q

What type of split is seen in pemphigus vulgaris?

A

Intrapidermal split.

42
Q

What type of split is seen in bullous pemphigoid?

A

Subepidermal split.

43
Q

What is the clinical presentation of pemphigus vulgaris?

A

Skin involvement as many flaccid blisters that easily rupture, leaving weeping and crusting erosions; mucous involvement, commonly oral, presents as ulcers.

44
Q

What is a positive sign associated with pemphigus vulgaris?

A

Positive Nikolsky’s sign.

45
Q

What is acantholysis?

A

Loss of intercellular connections, e.g., desmosomes, causing loss of cohesion between keratinocytes.

46
Q

What are acantholytic keratinocytes?

A

Round-shaped cells with desmosomal spines, called ‘prickle cells’.

47
Q

What does acantholysis cause?

A

Intrapidermal vesicles to form (clear spaces between acantholytic keratinocytes).

48
Q

What is pemphigus foliaceus?

A

A rarer form of pemphigus where only Dsg 1 is targeted, resulting in only skin involvement and no mucosal involvement.

49
Q

What are the characteristics of skin involvement in pemphigus foliaceus?

A

Involves superficial, smaller blisters that quickly rupture to form crusting erosions.

50
Q

What is paraneoplastic pemphigus?

A

A very rare malignancy-associated disorder characterized by painful oral erosions and a polymorphic inflammatory rash.

51
Q

What is paraneoplastic pemphigus usually associated with?

A

Usually associated with lymphoproliferative neoplasm.

52
Q

What is the gold standard investigation for pemphigus?

A

DIF microscopy from perilesional skin biopsy, which shows IgG autoantibodies deposited on the surface of keratinocytes in and around lesions.

53
Q

What does tissue biopsy histopathology show in pemphigus vulgaris?

A

Intraepidermal vesicles with acantholysis, characterized by a ‘chicken-wire’ or ‘fish-net’ appearance.

54
Q

What does the ELISA assay identify in pemphigus?

A

It can identify if Dsg 3 or Dsg 1 autoantibodies are present to distinguish between forms.

Clinical lectures: Yr2 (46 decks)

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