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Dermatology > Lecture 11: Bullae & Desquamation Disorders > Flashcards

Lecture 11: Bullae & Desquamation Disorders Flashcards

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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

Autoantibodies of the IgG class

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?

A

Lack of pruiritis

+ burning/pain

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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.

flaccid foliage

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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.
Had to google this, his slides seem weird here
Dsg3 means mucosal involvement

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21
Q

Pharm tx for Pemphigus

A
  • Prednisone 2-3mg/kg
  • Azathioprine/Mycophenolate mofetil

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?

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A

Chronic Steroid use

Might need Calcium/Vit D supplements

24
Q

What demographic is bullous pemphigoid typically seen in?

25
What is the underlying pathophysiology of Bullous pemphigoid?
Interaction of autoantibody with BP antigen.
26
What is the MC bullous autoimmune disease?
Bullous pemphigoid
27
What junction is disrupted in bullous pemphigoid?
Dermal-Epidermal Junction
28
What are the **most initial clinical findings** for bullous pemphigoid? (2)
* Pruiritis * Urticaria and papular lesions | Lasts weeks to months ## Footnote Prodrome period
29
Describe the classic cutaneous lesion of bullous pemphigoid and the result of its nikolsky sign (4)
* Large, tense, firm-topped bullae (not flaccid) * Serous or hemorrhagic fluid * Painful erosions after rupture * (-) Nikolsky **diff from pemphigous?**
30
Where does bullous pemphigoid tend to occur?
* Axilla * Medial thigh * Groin * Abdomen * Ventral forearm * Lower legs | No face or scalp as in pemphigus!
31
Are oral lesions seen in bullous pemphigoid?
Yes, but less painful and severe.
32
Gold standard for diagnosing bullous pemphigoid?
Biopsy of **perilesional skin** showing linear IgG deposits or C3 along basement membrane.
33
What two serologic studies can be done to diagnose bullous pemphigoid?
* DIF showing IgG antibodies (70% of pts) * ELISA for BPAG1 and BPAG2 (highly sens and specific, but expensive?)
34
Most important part of treating bullous pemphigoid
Refer to derm
35
How is bullous pemphigoid treated?
Prednisone 50-100mg/d + Azathioprine 150mg/d | Taper once clearance starts, same as pemphigus?
36
Why is osteoporosis sometimes also seen in bullous pemphigoid?
Chronic steroid use
37
What is the underlying pathophysiology of erythema Multiforme?
Acute HSR reaction affecting skin + mucous membranes | **Type IV HSR**
38
MCC of Erythema Multiforme
HSV
39
What would you expect to see in erythema multiforme?
* Erythematous, papular or urticarial lesions * Bullae later (small or large) * Bullae can contain serous OR hemorrhagic fluid, presenting as both **pruiritic and painful**
40
How do mucosal lesions tend to present in Erythema Multiforme?
* Erosions and Ulcerations * **Specifically in the eyes, can present as corneal ulcers or anterior uveitis**
41
T/F Erythema Multiforme does not present with constitutional symptoms?
False: presents with fever, weakness, malaise, and fatigue
42
T/F: Erythema Multiforme is bilateral and symmetrical?
True
43
What characterizes MINOR erythema multiforme?
* Little to **NO mucosal involvement** * (+) vesicles, but **NO bullae** * **NO systemic symptoms** * **NO spread past face and extremities**
44
How is MINOR erythema multiforme treated?
* Antihistamines for pruiritis * Topical low-dose steroids * Antivirals if 2/2 HSV (Valacyclovir) * Oral lesions require **High dose steroid gel**
45
What are the 3 ways to treat painful oral lesions in Erythema Multiforme?
* High dose steroid gel (fluocinonide gel 2-3x/d) * Compound topical oral solution (Magic swizzle) * 1:1 viscous lido/benadryl/Maalox/(+/-) dexamethasone **Swish, gargle, spit** | DO NOT SWALLOW
46
Tx of mucocutaneous lesions in MAJOR erythema multiforme?
* 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
What helps with recurrent erythema multiforme?
Daily antivirals | But could result in secondary bacterial infection
48
What is the underlying pathophysiology of SJS & TEN?
Cytotoxic event destroying keratinocytes
49
MCC of SJS & TEN?
DRUGS
50
What physical exam finding might occur **prior to the massive desquamation** of SJS & TEN?
Skin tenderness | Generally they are extremely sick looking
51
What are the characteristics of the mucocutaneous lesions seen in SJS/TEN?
* Target lesions * Rapid confluence * (+) Nikolsky sign
52
If SJS spreads and causes the loss of hair and nails, what is it now?
TEN | Idk he put in parentheses
53
At what point is SJS/TEN an emergency?
* Fever * HR > 120 BPM * Sloughing of epidermis
54
Classify SJS, SJS/TEN, and TEN
* SJS = < 10% BSA * SJS/TEN = 10-30% BSA * TEN = > 30% BSA | S before T
55
How is SJS diagnosed?
Clinically
56
How is SJS/TEN managed?
* 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
What do you want to give to SJS/TEN early on in tx?
* IV Steroids * IVIG

Dermatology (12 decks)

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