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Dermatology BOLOGNIA > 30- Pemphigoid group > Flashcards

30- Pemphigoid group Flashcards

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1
Q

which atoantibodies is bullous pemphigoid associated with?

A 
BP 180 (transmembrane protein)
BP 230 (cytoplasmic protein belonging to the plakin family)
  • almost all patients with BP ahave circulating IgG autoantibodies that bind to BP180.
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2
Q

Which domain region of BP180 do the IgG autoantibodies in bullous pemphigoid bind to?

A
  • the NC16A domain.
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3
Q

which domain of the BP230 region does the IgG in bullous pempghiod bind to?

A

C terminal region.

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4
Q

What autoantigens are found in gestational pemphigoid?

A
  • BP 180 and 230
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5
Q

What autoantigens are found in mucous membrane pemphigoid?

A

BP 180
BP 230
Laminin 332, Laminin 311 and Integrin B4

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6
Q

What autoantigens are found in linear IgA?

A
  • LAD antigen
  • BP 180
  • BP 230
  • Type VII collagen
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7
Q

what autoantigens are found in epidermolysis bullosa acquisita?

A

type VII collagen

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8
Q

What autoantigens are found in bullous systemic lupus erythematousus?

A

type VII collagen

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9
Q

what are the clinical features of bullous pemphigoid?

A

Non bullous pemphigoid:

  • polymphoorphic, non specific featres
  • mid to severe intractable pruritis

Bullous phase:

  • development of vesicles and bullae on normal or erythematous skin + urticated plaques.
  • blisters may be blood tinged.
  • in 10-30% of patients may have oral cavity involvement.
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10
Q

what diseases are associated with bullous pemphigoid?

A
  • rarely Inflammatory bowel disease
  • Hashitmoto thryoiditis
  • rheumatoid arthritis
  • dermatomyositis
  • lupus erythematosus
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11
Q

what is the epitope spreading phenomenon?

A
  • chronic inflammatory process at the dermal-epidermal junction which results in exposure of antigens to autoreactive T lymphocytes which leads to a secondary immune response.
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12
Q

What neurological disorders is BP associated with/

A

Parkinson disease, dementia, psychiatric disordrs like bipolar and unipolar disorders, stroke.
Multiple sclerosis.

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13
Q

What drugs are implicated in bullous pemphigoid?

A
  • diuretics
  • NSAIDs
  • antibiotics
  • ACE inhibitors
  • TNF inhibitors
  • checkpoint inhibitors like pembrolizuma.
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14
Q

what are the histological features of bullous pemphigoid?

A
  • subepidermal blister
  • dermal inflammatory infiltrate with eosinophils in th uppdr dermis
  • net of fibrin
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15
Q

What are the features of direct immunoflurescence microscopy of BP

A
  • linear deposits of IgG +/- C3 along the basement membraane

Ig1 and Ig4 are the predominant subclasses.

n-serrated pattern

salt split skin: deposits at the roof

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16
Q

What linear fluorescence pattern is seen at the basement membrane zone?

A

n serrated: bullous pemphigoid and linear IgA.

u serated: epidermolysis bullosa acquisita.

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17
Q

What are some differential diagnosis of prebullous pemphigoid.

A
  • drug reaction
    urticaria
    arthropod reaction
    scabies.
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18
Q

what are the differential diagnosis of bullous pemphigoid?

A
  • EBA
  • linear IgA
  • mucous membrane pemphigoid
  • pemphigoid incipiens
  • anti-p200 pemphigoids (neutrophils rather than eosinophils)
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19
Q

the presence of the which four clinical criteria strongly indicate a diagnosis of BP (in patients with subepidermal blistering)

A
  • absence of mucosal involvement
  • absence of skin atrophy
  • absence of head and neck involvement
  • age greater than70
20
Q

what is the prognosis of bullous pemphigoid?

A
  • 30% of patients have a relase in the first year
  • if therapy ceased then a relapse occurs within the first 3 months.
  • death rate is between 10 - 40%
21
Q

how is BP monitored

A
  • can be monitored by looking at the BP180 ELISA.

levels at day 0,60 and 150 appear to predict disease relapse (small decrease btween day 0 and 60 is associated with a relapse).

22
Q

What treatment options are available for mild and/or localized BP?

A
  • superpotent topical steroids
  • oral pred
  • doxy+ nicotinamide (500 - 2000 mg/day)
  • eryrhyomycin, penicilins
  • dapsone
  • topical immunomodulators
23
Q

What treatment options are available for severe bullous pemphigoid? (extensive disease > 10 blisters or inflammatory lesions/day)

A
  • superpotent topical steroids
  • oral pred 0.5-1 mg/kg /day
  • AZA
  • MMF (1.5 - 3 g/day)
  • MTX (7.5 mg-15 mg/week)
  • chlorambucil
  • IVIg
  • Rituximab
  • Omalizumab
24
Q

How long to patients with bullous pempigoid need to be treated for?

A

6 -12 monts (unless steroid-resistant)

25
Q

what is cicatricial pemphigoid?

A

this is a chronic, autoimmune SUBEPITHELIAL blistering disorder.
it involves the external mucosal surfaces and has a tendency to scar.

26
Q

who does cicatricial pemphigoid occur in?

A
  • usually in the elderly (between 60 -80 years of age)
  • women usually affected.
  • not associated with malignancy
27
Q

What is the pathogenesis of mucocutaneous pemphigoid?

A
  • binding of autoantibodies to the basement membrane zone of stratified epithelia.
    9bind to filament zone)
28
Q

what are the subgroups of mucocutaneous pemphigoid?

A
  1. anti-laminin 332 mucous membrane pemphigoid
  2. ocular mucous membrane pemphigoid
  3. lesions affect both mucosae and the skin.
    (anti BP antigen mucous membrane pemphigoid)
  4. variable involvement of mucosa without skin involvement.
29
Q

which side of the salt split skin does IgG autoantibodies bind to in anti-laminin 332 mucous membrane pemphigoid?

A

Dermal

30
Q

where do the autoantidbodies bind to in ocular mucous membrane pemphigoid?

A

B4 subunit of a6b4 integrin

31
Q

what are the two most frequent sites of involvement for mcous membrane pemphigoid

A
  • oral and conjunctaival mucosae.
32
Q

what are the clinical features of mucous membrane pemphigoid?

A
  • disease affects all mucosal sites including genitals, anus, aerodigestive tract/oesophagus.
  • 85% of patients will have ora involvement.
  • after healing lesions may have white reticulated striations
  • nasopharangal, eye, mouth involvement.
  • oesophageal disease may present as dysphagia or it may be asymptomatic.
  • involvement of genital and anal mucosa is rare.
  • skin involved in 25-30% of patients: busually scalp, face, neck and upper trunk ar involved.
33
Q

how do occular manifestations of cicatricial pemphigoid present?

A
  • conjunctiva commonly affected.
  • usually both eyes commonly affected but this can be unilateral.
  • symptoms may be burning, soreness, foreign-body sensation or mucus production.
34
Q

what are the complications of occular mucous membrane pemphigoid?

A
  • conjunctival fibrosis
  • trichiasis, entropion
  • shortened inferior fornices
  • symblepharon formation.
35
Q

how does nasopharangeal invovlement in mucous membrane pemphigoid manifest?

A
  • crustd ulcerations, epistaxis, fibrous adhesions between adjacent mucosal surfaces
  • airway obstruction
  • pharangeal involvement presents as ulceration of posterior or lateral pharynx and dysphagia.
  • hoarsness, loss of speach.
36
Q

what treatment options are there for mucus membrane pemphigoid?

A
  • topical corticosteroids (mouthwashes or topical preparations)
  • corticosteroid sprays/ inhalers
  • Dapsone 50 mg- 150 mg daily
  • cylophsphamide 1-2 mg/kg/day for the treatment of rapidly progressing disease.
  • AZA and MMF are also elpfus.
  • pred is less effective for mucosal disease.
37
Q

What is Brunsting-Perry syndrome?

A

Brungsting-Perry is a rare-variant of mucous membrane pemphigoid affecting the head an neck.

  • appears as urticated plaques which blister around the edge.
38
Q

What autoantibodies cause Epidermolysis Bullosa Acquista?

A
  • autoantibodies to type VII collagen (located in the lamina densa and sublamina densa region).
39
Q

What are the clinical features of EBA?

A

Acral blistering that heals with atrophic scarring, milia formation and dyspigmentation.

cutaneous blisters which may becomine hemaorrhagic and erosions which appear within non-inflammed skin or areas of scarring.

there is scalp involvement in up to 20% of patients.

inflammatory EBA can present as pruritic vesiculobullae on an erythematous base.

May also have a mucous membrane pemphigoid-like presentation, Brungstin-Perry presenation and a Linear IgA bullous dermatosis-like presentation.

childhood EBA may have an overp with childhood BP and linear IgA.

40
Q

What diseases are associated with EBA?

A
  • inflammatory bowel disease, myelsoma, systemic lupus erythematosus, rheumatoid arthritis, thryoiditis and diabetes mellitus.
41
Q

what is the histology of epidermolysis bullosa acquisita?

A
  • in the mechanobullous form(subepidermal blistering)

- dermal-epidermal cleavage

42
Q

what findings are presents in EBA on electron microscopy.

A
  • dermal-epidermal cleavage
  • sublamina densa zone.

(cleavage plan is not always a reliable criterion for dicriminating EBA from other subepidermal bullous disorders)

43
Q

What does is seen on DIF in epidermolysis bullosa acquisita?

A
  • IgG deposits in a continuuos linear pattern along the epidermal basmeent membrane zone.

C3,IgA,IgM amybe found

in salt-split skin - deposits located ont eh dermal side of the cleavage.

u-serrated immunodeposition pattern at the basmenet membrane zone is seen.

EBA binds to 290 kDA and 145 kDa protein in Western immunoblotting studies.

44
Q

What are the differential diagnosis for EBA?

A
  • inherited dystrophic epidermolysis bullousa
  • bullous pemphigoid
  • mucous membrane pemphigoid
  • porphyria cutanea tarda
  • psoduoporphyria
  • porphyria variegata
45
Q

What treatment options are available for EBA?

A
  • treatment is difficult

Colchicine, dapsone, gold and cyclosporin can be beneficial.

cyclophosphamide, AZA, MTX, MMF are useful for BP-like variant of EBA.

IVIg may be helpful for resistant cases.

Dermatology BOLOGNIA (20 decks)

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