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Dermatology 2 > Vesiculobullous Disease > Flashcards

Vesiculobullous Disease Flashcards

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Dermatology Board Prep flashcards
1
Q

List the 2 vesiculobullous diseases

A
  1. Bullous Pemphigoid

2. Pemphigus Vulgaris

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2
Q

Define vesiculobullous disease

A
  • Uncommon chronic skin disorders caused by autoantibodies against various cutaneous proteins
  • Significant quality of life issues
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3
Q

Epidemiology of bullous pemphigoid (BP)

A
  • Bullous pemphigoid occurs equally in males and females.
  • More prevalent in elderly patients.
  • Usually in patients > 65 years of age with other comorbidities
  • Median age of onset is 68-82
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4
Q

Causes of BP

A
  • A drug, an injury, or skin infection can trigger the onset of disease
  • There are HLA associations indicating genetic predisposition to the disease
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5
Q

Drug causes of BP

A
  • furosemide
  • captopril
  • penicillamine
  • antibiotics
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6
Q

Pathophysiology of BP

A
  • Bullous pemphigoid is associated with the production of autoantibodies (IgG, +/- IgE immunoglobulins) targeting the basement membrane
  • The binding of the autoantibodies to the proteins at the basement membrane stimulates destructive inflammatory cascade, predominantly mast cells and eosinophils
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7
Q

Name the 2 BP antigens that are autoantibody targets

A

Antibodies directed against 2 antigens: BP180 and BP230

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8
Q

Why is the basement membrane important in BP?

A

The basement membrane is important for the adhesion of the epidermis to the dermis, and so when targeted, leads to a separation (blister) in this space

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9
Q

What is the result of autoantibodies targeting the basement membrane in BP?

A

Results in the separation of the epidermis from the dermis forming a subepidermal blister

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10
Q

How does BP present on clinical exam?

A
  • Prodromal erythematous, pruritic urticarial or papular eruption.
  • Pruritus can be severe**
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11
Q

Where are the lesions with BP?

A
  • Lesions usually bilateral and symmetric

- Predilection for groin, axillae, trunk, legs and flexural forearms.

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12
Q

What is the timeline for BP lesions?

A

-Evolves in weeks to months to bullae: small expanding to large, tense bullae, which rupture forming erosions.

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13
Q

Are the mucous membranes involved in BP?

A
  • yes, but involvement rare - less than 10-20%

- if there is involvement, generally oral cavity.

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14
Q

Dx of BP

A
  • Diagnosis is made based upon clinical, histologic, and IF features
  • Most cases diagnosis confirmed by a skin biopsy (2 kinds: H&E + DIF)
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15
Q

Explain the H&E biopsy for BP

A
  • biopsy of lesional skin (involved skin)
  • report would reveal: “subepidermal blister with an inflammatory cell infiltrate containing eosinophils in the superficial dermis the presence of C3 deposition along the epidermal basement membrane zone”
  • used for the confirmation of the diagnosis
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16
Q

Explain the DIF biopsy for BP

A
  • Direct immunofluorescence (DIF) staining of normal-appearing perilesional skin (uninvolved skin)
  • Reveals linear IgG &/or complement C3 along basement membrane
17
Q

What other test for dx of BP?

A

ELISA test for detection of circulating antigen specific, BP180 and BP230,IgG and IgE antibodies is available.

18
Q

What is involved with DIF biopsy?

A
  • Refrigerated in Michel’s media
  • The antibodies IgG fluoresce neon green for pathologist
  • Necessity in diagnosis of blistering diseases
  • If perilesional (noninvolved skin) sample is taken, recommend > 3.0 mm away from blister edge
19
Q

Tx of bullous pemphigoid

A
  • treatment is directed at reducing the inflammatory response and autoantibody production*
  • Goal of therapy is to decrease blister formation
  • Use immunosuppressing systemic therapy
  • Promote healing of blisters and erosions (wound care, prevent /treat secondary infections)
  • Determine the minimal dose of medication necessary to control the disease process.
  • Clear patient of blisters with high dose medication, then slow taper
20
Q

For specific bullous pemphigoid tx…

A

See Dr. Letassy’s handout :)

21
Q

Prognosis of BP

A
  • Characterized by a waxing and waning course
  • Occasional spontaneous remission in the absence of treatment
  • Localized disease often resolves spontaneously, but spontaneous remission can even occur in patients with more generalized disease
  • One study reported that 8 of 30 adults with bullous pemphigoid went into remission after approximately 15 months (range, 3–38 months) of active disease
22
Q

Pemphigus Vulgaris (PV) epidemiology

A
  • Equal frequency in men and women
  • Usually 5th and 6th decades (slightly younger than BP)
  • MC in Jewish and those of Mediterranean descent and from Middle East
23
Q

Etiology of PV

A

Autoimmune blistering disease with IgG autoantibodies directed against desmogleins 3 and 1

24
Q

What are desmogleins?

A

desmosomal proteins that help the keratinocytes in the epidermis attach to each other

25
Q

Which PV lesions dominate with autoantibodies towards Dsg3?

A

mucosal lesions dominate

26
Q

Which PV lesions dominate with autoantibodies towards both Dsg1 and 3?

A

mucocutaneous disease

27
Q

What do autoantibodies towards Dsg1 and 3 cause in PV?

A

**acantholysis – separation of the skin

28
Q

Clinical presentation of PV

A
  • Characterized by painful mucosal erosions and thin-walled (i.e. more flaccid, not as tense), easily ruptured bullae
  • Blisters typically arise on normal-appearing skin but can be erythematous skin
  • *No preceding urticarial stage line in BP
  • Because PV blisters are fragile, MC for skin lesions observed to be erosions resulting from broken blisters
29
Q

Clinical findings in PV

  • sign
  • location of 1st involvement
A

+ Nikolsky sign – epidermis easily shears away from underlying dermis with pressure, twisting or rubbing
-usually appears first in the mouth (60% cases) or at site of skin injury

30
Q

Other mucosal involvement with PV

A

Mucosal involvement usually oral cavity but may involve nasal mucosa, pharynx, larynx, esophagus, conjunctiva, and genitals

31
Q

Define pemphigus foliaceus

A
  • no mucosal lesions; scaly, crusted lesions on an erythematous base
  • Also calledsuperficial pemphigus as occurs higher in epidermis
32
Q

Dx of PV

A
  • Made by histology from lesional skin + confirmatory immunochemical study via direct immunofluorescence or Enzyme-Linked Immunosorbent Assay
  • Histology shows acantholysis with intraepidermal blister formation
33
Q

What does PV show on DIF?

A

staining reveals “chicken wire” pattern of intercellular IgG deposited in perilesional skinbound to the cell surface of keratinocytes

34
Q

ELISA for PV

A

Autoantibodies against desmoglein 3 &/or, desmoglein 1

35
Q

PV prognosis

A
  • Prior to glucocorticoid therapy, PV was almost always fatal from malnutrition, dehydration and sepsis
  • Still a disease associated with a significant morbidity and mortality, now approximately 10% or less mortality
  • Infection +/- immunosuppression of tx is often the cause of death
  • Remission is possible +/- relapses
36
Q

Tx of PV

A
  • No FDA approved treatments
  • Approach to tx varies widely
  • Topical steroids may suffice for localized dz
  • Systemic glucocorticoids is mainstay; 2.0 mg/kg/day single daily dose
  • Once disease activity is controlled, taperingprednisone to as low a dose as possible is the goal
37
Q

Tx of PV continued…

A
  • If continued relapses with daily prednisone dose of 10 mg or higher, adjunctive immunosuppressive agents should be considered
  • Mucosal disease may benefit from the use of glucocorticoid elixirs as a swish and spit or dental trays to help apply class I corticosteroid gels or ointments to the gingiva
38
Q

Considerations when using immunosuppressive therapy for PV

A
  • Monitor all patients closely for potential side effects- blood count, liver and kidney laboratory abnormalities, gastrointestinal ulcer disease, high blood pressure, diabetes, glaucoma, cataracts, osteoporosis, and infection
  • Consider potential incidence of malignancies that might be associated
  • Consider risks of infertility and teratogenicity
39
Q

For specific PV tx…

A

See Dr. Letassy’s handout :)

Dermatology 2 (10 decks)

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