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derm > Lecture 11: Bullae & Desquamation Disorders > Flashcards

Lecture 11: Bullae & Desquamation Disorders Flashcards

1
Q

What adheres the epidermis to the dermis?

A

Hemidesmosomes

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2
Q

What is the underlying pathophysiology for pemphigus?

A
  • loss of cell to cell adhesion in the epidermis
  • 2/2 Autoantibodies of the IgG class that attatch to desmogleins and inhibit desmosomes from aiding in cell adhesions (autoimmune disease)

Results in acantholysis

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3
Q

What are the two main descriptors of pemphigus vulgaris?

A
  • Flaccid blisters on skin
  • Erosions on mucous membranes
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4
Q

What specific demographic is MC for pemphigus vulgaris?

A

Jewish/Mediterranean descent

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5
Q

What characterizes pemphigus foliaceus specifically?

A

Scaly and crusted lesions

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6
Q

What demographic is MC for Pemphigus Foliaceus?

A

Brazilian

Brazil has lots of foliage

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7
Q

When does pemphigus tend to occur in general age-wise?

A

40-60

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8
Q

How are the lesions of pemphigus spaced?

A

Scattered and discrete with a random pattern

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9
Q

T/F: Pemphigus vesicles and bullae are filled with blood?

A

False: They are filled with serous content

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10
Q

T/F: Pemphigus blisters are flaccid and easily ruptured, often described as weeping?

A

True

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11
Q

What is a nikolsky sign?

A

Dislodging or normal appearing epidermis by lateral finger pressure in the vicinity of lesions.

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12
Q

Where is pemphigus vulgaris MC found on the body?

A
  • Scalp
  • Face
  • Chest
  • Axillae
  • Groin
  • Umbilicus
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13
Q

Where does Pemphigus Vulgaris typically begin?

A

Oral mucosa

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14
Q

What happens to a person due to painful mouth lesions in pemphigus vulgaris?

A

Inadequate food intake

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15
Q

What specific symptom is not seen in pemphigus vulgaris mouth lesions? What other symptoms are seen instead?

A

They do not have pruritus!

  • burning/pain
  • epistaxis
  • dysphagia
  • weakness
  • malaise
  • weight loss

lots of SE cause not eating which causes more SE

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16
Q

What is a key distinguishing factor seen on Physical exam between pemphigus vulgaris and foliaceus?

A

Lack of mucosal involvement in foliaceus

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17
Q

How does pemphigus foliaceus typically present?

A

Flaccid bullae that quickly rupture, resulting in superficial erosions.

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18
Q

What extends to more of the body: Pemphigus vulgaris or foliaceus?

A

Vulgaris, which goes down to the groin region.

Foliaceus is only face, scalp, upper chest, and abdomen

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19
Q

How do you test for pemphigus?

A
  • Biopsy at the edge of a blister showing (+) deposits of IgG
  • Direct immunofluorescence staining (DIF) of normal appearing skin adjacent to a lesion showing IgG and C3.

You must do a biopsy + DIF/ELISA

ELISA can be done but very expensive.

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20
Q

What ELISA results correspond to Pemphigus Vulgaris? Foliaceus?

A
  • (+) a-Dsg3 = PV
  • (+) a-Dsg1 = PF

PV can have neg or pos Dsg1, but only 3 matters.

V is later in the alphabet, so higher desmoglein.

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21
Q

Pharm tx for Pemphigus

A
  • Prednisone 2-3mg/kg
  • Azathioprine or Mycophenolate mofetil (Cellcept)

Both

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22
Q

When do you D/C prednisone for pemphigus tx?

A

Cessation of new blister formation and disappearance of Nikolsky sign.

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23
Q

Why can osteoporosis occur in pemphigus?

A

Chronic Steroid use

Might need Calcium/Vit D supplements

24
Q

What demographic is bullous pemphigoid typically seen in?

A

Elderly

25
Q

What is the underlying pathophysiology of Bullous pemphigoid?

A

Interaction of autoantibody with BP antigen.

26
Q

What is the MC bullous autoimmune disease?

A

Bullous pemphigoid

27
Q

What junction is disrupted in bullous pemphigoid?

A

Dermal-Epidermal Junction

28
Q

What are the most initial clinical findings for bullous pemphigoid? (2)

A
  • Pruiritis
  • Urticaria and papular lesions

Lasts weeks to months

Prodrome period

29
Q

Describe the classic cutaneous lesion of bullous pemphigoid and the result of its nikolsky sign (4)

A
  • Large, tense, firm-topped bullae
  • Serous or hemorrhagic fluid
  • Painful erosions after rupture
  • (-) Nikolsky
30
Q

Where does bullous pemphigoid tend to occur?

A
  • Axilla
  • Medial thigh
  • Groin
  • Abdomen
  • Ventral forearm
  • Lower legs

No face or scalp as in pemphigus!

31
Q

Are oral lesions seen in bullous pemphigoid?

A

Yes, but less painful and severe.

32
Q

Gold standard for diagnosing bullous pemphigoid?

A

Biopsy of perilesional skin showing linear IgG deposits or C3 along basement membrane.

33
Q

What two serologic studies can be done to diagnose bullous pemphigoid?

A
  • DIF showing IgG antibodies (70% of pts)
  • ELISA for BPAG1 and BPAG2 (highly sens and specific, but expensive?)
34
Q

Most important part of treating bullous pemphigoid

A

Refer to derm

35
Q

How is bullous pemphigoid treated?

A

Prednisone 50-100mg/d + Azathioprine 150mg/d

Taper once clearance starts

36
Q

Why is osteoporosis sometimes also seen in bullous pemphigoid?

A

Chronic steroid use

37
Q

What is the underlying pathophysiology of erythema Multiforme?

A

Acute HSR reaction affecting skin + mucous membranes

Type IV HSR

38
Q

MCC of Erythema Multiforme

A

HSV

39
Q

What would you expect to see in erythema multiforme?

A
  • Erythematous, papular or urticarial lesions
  • Bullae later (small or large)
  • Bullae can contain serous OR hemorrhagic fluid, presenting as both pruiritic and painful
40
Q

How do mucosal lesions tend to present in Erythema Multiforme?

A
  • Erosions and Ulcerations
  • Specifically in the eyes, can present as corneal ulcers or anterior uveitis
41
Q

T/F Erythema Multiforme does not present with constitutional symptoms?

A

False: presents with fever, weakness, malaise, and fatigue

42
Q

T/F: Erythema Multiforme is bilateral and symmetrical?

A

True

43
Q

What characterizes MINOR erythema multiforme?

A
  • Little to NO mucosal involvement
  • (+) vesicles, but NO bullae
  • NO systemic symptoms
  • NO spread past face and extremities
44
Q

How is MINOR erythema multiforme treated?

A
  • Antihistamines for pruiritis
  • Topical low-dose steroids
  • Antivirals if 2/2 HSV (Valacyclovir)
  • Oral lesions require High dose steroid gel
45
Q

What are the 3 ways to treat painful oral lesions in Erythema Multiforme?

A
  • High dose steroid gel (fluocinonide gel 2-3x/d)
  • Compound topical oral solution (Magic swizzle)
  • 1:1 viscous lido/benadryl/Maalox/(+/-) dexamethasone Swish, gargle, sit

DO NOT SWALLOW

46
Q

Tx of mucocutaneous lesions in MAJOR erythema multiforme?

A
  • IVF
  • Oral compound solution (Magic swizzle)
  • Systemic high dose prednisone if severe
  • Pain control
  • Wet compresses via Burow’s solution for large erosions
  • ANY OCULAR = consult oph
47
Q

What helps with recurrent erythema multiforme?

A

Daily antivirals

But could result in secondary bacterial infection

48
Q

What is the underlying pathophysiology of SJS & TEN?

A

Cytotoxic event caused by immune response destroying keratinocytes

49
Q

MCC of SJS & TEN?

A

DRUGS

50
Q

What physical exam finding might occur prior to the massive desquamation of SJS & TEN?

A

Skin tenderness

Generally they are extremely sick looking

51
Q

What are the characteristics of the mucocutaneous lesions seen in SJS/TEN?

A
  • Target lesions
  • Rapid confluence
  • (+) Nikolsky sign
52
Q

If SJS spreads and causes the loss of hair and nails, what is it now?

A

TEN

Idk he put in parentheses

53
Q

At what point is SJS/TEN an emergency?

A
  • Fever
  • HR > 120 BPM
  • Sloughing of epidermis
54
Q

Classify SJS, SJS/TEN, and TEN

A
  • SJS = < 10% BSA
  • SJS/TEN = 10-30% BSA
  • TEN = > 30% BSA

S before T

55
Q

How is SJS diagnosed?

A

Clinically

56
Q

How is SJS/TEN managed?

A
  • IVF
  • Parenteral nutrition
  • IV pain control
  • Wound care via wet dressing with Burow’s
  • Non-adherent dressings for the eye made of saline and erythromycin ointment
57
Q

What do you want to give to SJS/TEN early on in tx?

A
  • IV Steroids
  • IVIG

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