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Dermatology BOLOGNIA > 29- Pemphigus > Flashcards

29- Pemphigus Flashcards

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1
Q

What are the three major forms of pemphigus?

A
  • pemphigus foliaceus
  • pemphigus vulgaris
  • paraneoplastic pemphigus
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2
Q

What is the mean age of onset for pemphigus foliaceus and pemphigus vulgaris?

A

50 - 60 years old

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3
Q

What are the features of fogo selvagem?

A
  • variant of pemphigus foliaceus.
  • common in rural Brazil
  • affects young adults and children in rural areas.
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4
Q

Which autoantibodies are found in pemphigus?

A
  • IgG autoantibodies against cell surface of keratinocytes.
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5
Q

why do neonates with mothers who have pemphigus vulgaris get transient disease?

A
  • Maternal IgG crosses the placenta.
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6
Q

What are the target antigens in pemphigus vulgaris?

A

Desmoglein 1 and 3 in the mucocutaneous type and

desmoglein 3 in the mucosal dominant type.

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7
Q

What are the target antigents in pemphigus foliaceus?

A

IgG autoantibodies to desmoglein 1

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8
Q

What are the target antigents in paraneoplastic pemphigus?

A

Desmoglein 1 , Desmoglein 3, Plectin, Epiplakin, Desmopakin I, Desmoplakin II, BPAG1, Envoplakin, Periplakin, A2ML1

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9
Q

Do neonates develop blisters in mothers who have pemphigus foliaceous?
Why or why not?

A

Neonates do not develop blisters in mothers with pemphigus foliaceus (As opposed to mothers with pemphigus vulgaris).

This is because the distribution
of Dsg 3 is found through out the epidermis Therefore the pemphigus
foliaceus sera containing only anti-Dsg1 IgG cannot induce blisters in neonatal skin.

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10
Q

Histologically, what other condition is indistinguisable from pemphigus foliaceus?

A
  • staph scalded skin syndrome

- bullous impetigo

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11
Q

What are the clinical features of pemphigus vulgaris?

A
  • painful erosions of the oral mucosa.
  • haemorrhagic crusts.
  • flaccid blisters and widespread cutaneous erosions.
  • there are 2 subgroups: mucosal-dominant and mucocutaneoustype.

erosions in the mouth are of different sizes with an irregular and ill-defined border.

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12
Q

What are the clinical features of pemphigus vegetans?

A
  • this is a variant of vegetative pemphigus vulgaris.
  • flaccid blisters that becoming erosions and then form fungoid vegitations in intertrigoues areas and on the scalp and face.
  • early lesions may present as pustules.
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13
Q

What are the 2 subtypes of pemphigus vegetans?

A
  • Neumann type

- mild Hallopeau type

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14
Q

What are the clinical features of pemhpigus foliaceus?

A
  • scaly crusted cutensoud erosions on an erythematous base.
  • no clinically apparent mucosal involvement
  • seborrheic in distrubution
  • oral manifestations are rare.
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15
Q

What are the clinical features of Senear-Usher syndrome?

A
  • localized variant of pemehigus foliaceus.

- plaques in the malar region of the face and other seborrheic areas.

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16
Q

What are the clinical features of herpetiform pemphigus?

A
  • erythematous urticarial plaques

- tense vesicles presenting in a herpetiform arrangement.

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17
Q

What drugs induce pemphigus

A
  • penacillamine
  • captopril

*in patients recieving penicillamine pemphigus foliaceus is seen more commonly than pemphigus vulgaris.

18
Q

what is the proposed mehanism behind why penacillamine and captopril cause pemphigus?

A

The sulfhydryl groups in penacillamine interact with Dsg1 and Dsg3.

19
Q

What are the most commonly associated neoplastsm for paraneoplastic pemphigus?

A
  • non-Hodgkin lymphoma
  • chronic lymphocytic leukemia
  • Csaatleman disease
20
Q

What is the clinical presentation of paraneoplastic pemphigus?

A
  • intractable stomatitis
  • most patients have a severe pseudomembranous conjunctivitis.
  • polymorphic cutaneous findings including blisters, EMlike lesions and liechnoid eruptions.
  • occurence of blisters and erythema multiforme-like lesions on the pams and soles differentiates paraneoplastic pempigus from pempigus vulgaris.
21
Q

What respiratory features do patients with paraneoplastic pemphigus get>

A

bronchiolitis obilterans.

22
Q

What are the 2 distinct types of IgA pemphigus?

A
  • subcorneal pustular dermatosis type

- intraepidermal neutrophilic type

23
Q

What is the clinical presentation of IgA pemphigus?

A
  • flaccid vesicles or pusutls

- pusutls form annular or circinate pattern with crusts in the centre of the lesion.

24
Q

What condition is subcorneal pustular dermatosis type of IgA pemphigus clinically and histologically indistinguishable from?

A

Classic subcornal pustular dermatosis.

25
Q

What is seen on DIF in IgA pemphigus?

A

IgA deposition on the cell surfaces of epidermal keratinocytes

IgA autoantibodies react with upper epidermal surfaces in subcorneal psutlar dermatosis type .

in the intraepiermal enturophillic type - IA autoantibodies foudn in the entire epidermis.

26
Q

What subclass of IgA autoantibodies if found in IgA pemphigus?

A
  • IgA1.
27
Q

What are the characteristic histologic findings of pemphigus?

A
  • acantholysis
    -intraepidrmal blistr formation
    acantholysis above the basal layer
    -“rounded keratinocytes’
28
Q

What are the causes of an eosinophillic spongiosis?

A
  • pemphigus vulgaris
  • pemphigus foliaceous
    -BP
  • pemphigoid gestationis
  • mumcous membrane pemphigoid
  • Linear IgA bullous dermatoses
    Inset-bite reaction
    -drug eruptin
    -Id reaction
    -Wells sh dro e
    -PUPP
  • first stage of incontentia pigmenti
  • atopic dermatitis
  • contact dermatitis
    -MF
  • prurigo pigmentosa
    -Still disease
29
Q

What are the histological findings of paraneoplastic pemphigus?

A
  • variability in pemphigus vulgaris-like, EM-like, LP like histology.
30
Q

what does indirect immunoflurescence examine?

A

patient’s sera is examined inorder to demonstrate circulating IgG autoantibodies against epithelial cell surfaces.

31
Q

What is the recommended indirect immunoflurescence substrate for pemphigus vulgaris and pemphigus foliaceous?

A

Pemphigus vulgaris - monkey esophagus.

pemphigus foliaceous - human skin/ ginea pig esophagus (anti Dsg1)

32
Q

What is the recommended indirect immunoflurescence substrate for paraneaoplastic pemphigus?

A

monkey esophagus.
guinea pig esophagus (anti Dsg1)

and rat bladder

33
Q

What is the recommended indirect immunoflurescence substrate for bullous pemphigoid, Linear IgA bullous dermatoses

A

Human skin - salt split

34
Q

What is the recommended indirect immunoflurescence substrate for mucous membrane pemphigoid?

A

human skin - salt-split, normal oral or genital mucosa.

35
Q

What are the differential diagnosis for mouth ulcerations?

A
  • acute herpetic stomatitis
    -aphthous stomatitis
    -EM/SJS,
    lichen planus,
    systemic lupus
    mucous membrane pemphigoid.
  • Hailey-Hailey disease
36
Q

What are the differential diagnosis of pemphigus foliaceus?

A
  • subcorneal pustular dermatosis, subacute cutaneous LE and seborrhic dermtatitis, bu,llous impetigo.
37
Q

What is the treatment ladder for pemphigus vulgaris?

A
  • oral prednisone 1mg/kg/day
  • Azathioprine
  • MMF
  • CsA
  • pulsed methyl pred
  • cyclosporine
  • MTX
  • plasmapheresis
    high dose IVIg
  • Rituximab
  • Extracoporeal photopheresis.
38
Q

how does rituximab work in pemphigus?

A
  • induces a depletion of CD20+ B cells and a decline in IgG.

- also decreases desmoglein-specific T cells.

39
Q

What is the treatment for paraneoplastic pemphigus?

A
  • removal of tumours like thymomas or Castelman diseases. It may take 6-18 months to see complete resolution of lesions.
40
Q

What is the treatment for IgA pemphigus?

A

Dapsone is the drug of choice.

Clinical response seen within 24-48 hours.

Sufapyridine and acitretin are useful alternatives.

Dermatology BOLOGNIA (20 decks)

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