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Bolognia Book Club > Chapter 31 - Pemphigoid Group > Flashcards

Chapter 31 - Pemphigoid Group Flashcards

1
Q

What two antigens can be targets for bullous pemphigoid?

A

BP1(BP230) and BP2 (BP180)

*Note: these are both components of hemidesmosomes.

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2
Q

What is the most common region on BP180 that’s targeted by autoantibodies in bullous pemphigoid?

A

NC16A domain (non-collagenous 16A domain), which is located extracellularly but close to the transmembrane domain

*Note: additional antigenic sites exist within both the extracellular and intracellular domains of BP180.

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3
Q

Other than bullous pemphigoid, list five other autoimmune blistering disorders that produce subepidermal blisters.

A
  1. Gestational pemphigoid
  2. Cicatricial pemphigoid
  3. Linear IgA bullous dermatosis
  4. Epidermolysis bullosa acquisita
  5. Bullous systemic lupus erythematosus
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4
Q

Can bullous pemphigoid occur in children?

A

Yes, but only rarely

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5
Q

True or false: in about half of patients with bullous pemphigoid, a peripheral blood eosinophilia is noted.

A

True

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6
Q

True or false: systemic medications can lead to the development of bullous pemphigoid.

A

True

*Note: the list of implicated drugs is long.

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7
Q

True or false: in patients with bullous pemphigoid, circulating antibasement membrane antibodies will be present.

A

True; in 60-80% of patients circulating antibasement membrane antibodies can be detected (in addition to the antibodies deposited in the skin)

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8
Q

Where should a biopsy for DIF be taken if you suspect bullous pemphigoid?

A

Perilesional (i.e. unaffected) skin

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9
Q

What concentration of NaCl should be used to perform a salt split-skin test?

A

1 M NaCl

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10
Q

Can mucous membranes be affected in epidermolysis bullosa acquisita (EBA)?

A

Yes, just like in bullous pemphigoid (although it’s not super common)

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11
Q

True or false: epidermolysis bullosa acquisita (EBA) has a classic “non-inflammatory” form and an “inflammatory” form.

A

True; the “inflammatory” form closely resembles bullous pemphigoid

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12
Q

Which finding on DIF is associated with bullous pemphigoid: an “n-serrated” or “u-serrated” pattern?

A

“n-serrated” pattern (also associated with LABD)

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13
Q

What type of pattern on DIF is associated with epidermolysis bullosa acquisita?

A

“u-serrated” pattern

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14
Q

What enzyme should you test for before starting azithioprine?

A

Thiopurine methyltransferase

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15
Q

What enzyme should you test for before starting dapsone?

A

Glucose-6-phosphate

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16
Q

True or false: potent topical steroids appear to be as effective as oral steroids for the treatment of bullous pemphigoid.

A

True

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17
Q

Can cicatricial pemphigoid lead to blindness?

A

Yes

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18
Q

What two mucosal sites are most commonly affected by cicatricial pemphoid?

A

Oral and conjunctival mucosae

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19
Q

Symblepharon can occur in cicatricial pemphigoid. What’s symblepharon?

A

Fibrous tracts between the bulbar and palpebral conjunctival surfaces

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20
Q

Trichiasis can occur in cicatricial pemphigoid. What’s trichiasis?

A

Eyelashes that grow back towards the eye, causing irritation to the cornea or conjunctiva

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21
Q

True or false: BP usually heals with scars.

A

False; cicatricial pemphigoid heals with scars (hence the name) but bullous pemphigoid does not

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22
Q

True or false: patients with cicatricial pemphigoid have disease limited to the mucosal surfaces.

A

False; most of the time lesions are limited to the mucosal surfaces, but in 25-30% of cases, skin lesions also occur

*Note: these present as erythematous plaques, which become sites for blister formation and erosions, with subsequent atrophic scarring.

23
Q

What’s Brunsting-Perry pemphigoid?

A

A variant of cicatricial pemphigoid where mucosal involvement is absent or minimal, and there’s skin lesions limited to the head and neck. On the scalp, scarring results in cicatricial alopecia.

24
Q

True or false: the histologic features of cicatricial pemphigoid and bullous pemphigoid are very similar.

A

True; both show subepithelial blister formation and linear IgG/C3 on DIF

25
Q

Loss of vision is the most important complication of cicatricial pemphigoid, and life-threatening complications are rare. List one possible life-threatening complication.

A

Involvement of the larynx, trachea, or esophagus, leading to respiratory failure or inability to eat

26
Q

Systemic steroids are generally inadequate for treating cicatricial pemphigoid. What is the systemic treatment of choice for rapidly progressive or severe ocular disease?

A

Cyclophosphamide

27
Q

True or false: dapsone is a first-line therapy for controlling the oral and cutaneous lesions of cicatricial pemphigoid, however it is only appropriate for the treatment of mild ocular disease.

A

True; cyclophosphamide should be used to treat rapidly progressive or severe ocular disease

28
Q

What is the autoimmune target in epidermolysis bullosa acquisita?

A

Collagen VII

29
Q

Do mucous membrane lesions occur in epidermolysis bullosa acquisita?

A

Yes, they can, although involvement is variable

30
Q

The two clinical presentations of EBA are the inflammatory and non-inflammatory forms. Which diseases are mimicked in these two different presentations of the disease?

A
  • Inflammatory: mimics bullous pemphigoid or cicatricial pemphigoid
  • Non-inflammatory form: mimics dystrophic epidermolysis bullosa
31
Q

Will EBA have a “n-serrated” or “u-serrated” pattern on DIF of salt-split skin?

A

“u-serrated” pattern

32
Q

True or false: EBA tends to respond rapidly to systemic steroids.

A

False; EBA tends to be chronic and refractory, and reponds only occasionally to systemic steroids and systemic immunosuppressants

33
Q

What 2 self-Ag are directed against in BP?

A

BPAG2 (aka BP180, type XVII collagen) and BPAG1 (BP230); they are components of hemidesmosomes

34
Q

In Caucasians, which HLA class II alleles are associated with BP?

A

DQB1*0301. In Japanese patients, alleles are DRB1*04, *1101 and DQB1*0302

35
Q

Which domain of BPAG1 is bound by autoAb in BP?

A

NC16A domain (non-collagenous 16A domain)

36
Q

What is one of the major isotypes of anti-BPAG2 autoAb?

A

IgG4 subclass, which is regulated by Th2 cytokines, which themselves are produced by T lymphocytes

37
Q

When do you perform cancer screening in BP patients?

A

Systemic manifestations, atypical presentations, middle aged

38
Q

Which drugs have been implicated in drug-induced BP? Name 3 classes

A

Diuretics (furosemide), analgesics, D-penicillamine antibiotics

39
Q

Other than DIF, what other tests can be done for BP?

A

H&E, indirect DF, immunoelectron microscopy using gold, immunoblot, immunoprecipitation, ELISA

40
Q

What are the 4 clinical criteria that STRONGLY indicate BP?

A

1) no skin atropy, 2) no mucosal involvement, 3) no H&N involvement, 4) >70yo

41
Q

What is the treatment for BP?

A

Systemic corticosteroids (prednisone 0.5-1mg/kg/d, controls disease in 1-2wks, tapered over 6-9mths), superpotent topical steroids, +/- immunosuppressive drugs (MMF 1.5-3g/d, cyclosporine 1-5mg/kg/d, azathioprine, MTX, chlorambucil, cyclophosphamide). Remember to minimize complications (osteoporosis prophylaxis, gastric protection, assess cardiovascular function, infection)

42
Q

What is the pathogenesis of CP? Hint: there are 4 subgroups of autoAb

A

Tissue-bound (less often circulating) autoAb against components of the BMZ in stratified epithelia of mucosa and skin. 1) anti-laminin 5, 2) anti-beta4 of alpha6beta4 integrin (ocular CP), 3) anti-BPAG2 (anti-BP Ag mucosal pemphigoid, mucosa and skin), 4) ?auto-Ab mediated (variable mucosa involvement, no skin involvement)

43
Q

What are some complications of CP?

A

Blindness, periodontal ligament damage, teeth loss, adhesions, scarring (affecting conjunctiva, nasopharynx, esophagus, but rarely genitals/anus)

44
Q

What is the skin variant of CP?

A

Brunsting-Perry pemphigoid (skin lesions on H&N, minimal mucosal involvement)

45
Q

What is the treatment for CP?

A

Potent topical corticosteroids (mouthwash, gel, sprays, inhalers), tetracycline mouthwashes, good hygiene, intralesional steroids,

46
Q

When is systemic treatment required for CP?

A

Severe ocular, laryngeal, esophageal involvement, unresponsive to topical tx. Dapsone 50-150mg/d, cyclphosphamide 1-2mg/kg/d, azathioprine 2mg/kg/d (note: adjust as per thiopurine methyltransferase), +/- surgery

47
Q

What is the Ag targeted in epidermal bullosa acquisita?

A

Type VII collagen

48
Q

What are the “classical” clinical features of EBA?

A

Non-inflammatory, mechanobullous disease, acral blisters that heal with atrophic scarring/milia/hyper/hypopigmentation

49
Q

What are some complications of EBA?

A

Mitten deformity, syndactyly, nail dystrophy, nail loss, scarring alopecia

50
Q

What distinguishes EBA from inherited dystrophic epidermolysis bullosa?

A

Lack FHx, late onset, positive DIF findings

51
Q

What is the treatment for EBA?

A

Colchicine, systemic corticosteroids, azathioprine, MTX, cyclophosphamide, dapsone, gold, IGIv, syclosporine, extracorporeal photochemotherapy

52
Q

What are the different clinical variants in BP? Hint: there are 11 varients

A

Classical, pretibial pemphigoid, dyshidrosiform pemphigoid, vesicular pemphigoid, pemphigoid nodularis, erythrodermic BP, lichen planus pemphigoides, gestiational pemphigoid, childhood BP, vulvar childhood BP, drug-induced BP

53
Q

What are the predeominant IgG subclasses in BP?

A

IgG4, IgG1

54
Q
A

Bolognia Book Club (29 decks)

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