Bullae and Desquamation Disorders Flashcards

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1
Q

structures that work to adhere cells to one another

A

Desmosomes

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2
Q

proteins inside desmosomes to allow for normal desmosome formation

A

Desmogleins

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3
Q

what holds the epidermis together?

A

Hemidesmosomes join cells of the epidermal basement membrane to the basilar membrane

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4
Q
  • Autoimmune disorder
  • Loss of cell to cell adhesion in the epidermis
  • circulating antibodies of IgG that bind to desmogleins transmembrane glycoproteins in the desmosomes
  • Autoantibodies interfere with adhesion = acantholysis
A

pemphigus

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5
Q

2 types of pemphigus

A
  1. Pemphigus Vulgaris: flaccid blisters on skin and erosions on mucous membranes
  2. Pemphigus Foliaceus: scaly and crusted skin lesions
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6
Q

pemphigus MC in who?

A
  • Pemphigus Vulgaris - jewish/mediterranean
  • Pemphigus Foliaceus - brazil
  • 40-60 years old
  • M:F = incidence
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7
Q
  1. Vesicles and bullae w/ serous content
  2. Flaccid/easily ruptured weeping
  3. Arising on normal skin
  4. Scattered & discrete - Localized or generalized with random pattern; Extensive erosions = bleed easily
  5. Nikolsky Sign
A

pemphigus

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8
Q

Dislodging or normal appearing epidermis by lateral finger pressure in the vicinity of lesions
Pressure on bulla = lateral extension

what is this sign?
MC areas?

A

Nikolsky Sign

  1. Scalp
  2. Face
  3. Chest
  4. Axillae
  5. Groin
  6. Umbilicus
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9
Q

s/s of pemphigus vulgaris

A
  • Starts in oral mucosa
  • Months may elapse before actual skin lesions
  • burning/pain, No pruritus
  • Painful mouth lesions = inadequate food intake
  • Epistaxis
  • Hoarseness
  • Dysphagia
  • Weakness
  • Malaise
  • Weight loss
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10
Q

s/s of pemphigus foliaceus

A
  1. No mucosal involvement
  2. Cutaneous lesions
  3. initial lesions: flaccid bullae, quickly ruptures, leaving superficial erosion
  4. MC sites: face, scalp, upper chest, abdomen
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11
Q

w/u for pemphigus

A
  1. bx of edge of blister: (+) deposits of IgG
    - Direct immunofluorescence (IF) staining: IgG and C3 deposited in lesional and paralesional skin; performed on normal-appearing skin adjacent to a lesion
  2. ELISA for IgG ab to desmogelin 1 & 2
    - PF +a-Dsg1
    - PF + a-Dsg3 and Dsg 1
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12
Q

tx for pemphigus

A
  1. prednisone until no new blisters or nikolsky
  2. Concomitant Immunosuppressive: azathioprine / mycophenolate mofetil (Cellcept)
  3. wound care: wet compressions, routine bathing, anticipate infection
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13
Q

complications of pemphigus

A
  1. Fluid and electrolyte imbalances
  2. Secondary bacterial infections
  3. Osteoporosis - Calcium/Vit D supplementation
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14
Q
  • Rare autoimmune disease MC in elderly pts (60-80)
  • Interaction of autoantibody with BP antigen
  • MC bullous autoimmune disease
A

bullous pemphigoid

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15
Q

pathophys of bullous pemphigoid

A
  • BPAG1 & BPAG2 In hemidesmosomes of basal keratinocytes
  • Followed by complement and mast cell activation, attraction of neutrophils and eosinophils, release multiple bioactive molecules from inflammatory cells
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16
Q
  1. Prodrome (weeks - months): Pruritus, Urticaria and papular lesions
  2. large, tense, firm-topped bullae
  3. serous or hemorrhagic fluid
  4. painful erosions after rupture
  5. (-) Nikolsky
  6. MC sites: axilla, medial thigh, groin, abdomen, ventral forearm, lower legs
  7. Oral lesions: less severe and less painful than pemphigus
A

bullous pemphigoid

17
Q

w/u for bullous pemphigoid and findings

A
  1. Bx (Gold Standard): Linear IgG deposits along basement membrane
    - Perilesional skin
    - C3 may occur in absence of IgG
  2. DIF study: IgG ab present in 70% of pts
  3. ELISA - BPAG1 and BPAG2
    - highly sensitive and specific
    - few labs perform this test
18
Q

tx for bullous pemphigoid

A
  1. refer
  2. prednisone
  3. +/- azathioprine
  4. +/- topical steroids
19
Q

complications of bullous pemphigoid

A
  1. Fluid and electrolyte imbalances
  2. Secondary bacterial infections
  3. Osteoporosis - Calcium/Vit D supplementation
20
Q

Acute hypersensitivity reaction affecting the skin and mucous membranes
Sometimes eye involvement

A

erythema multiforme

21
Q

causes of erythema multiforme? which is MC?

A
  1. HSV (MC)
  2. Mycoplasma
  3. Sulfonamides
  4. PCN
  5. Allopurinol
  6. Barbiturates
  7. Phenytoin
  8. Phenylbutazone
22
Q

s/s of erythema multiforme

A
  1. Erythematous, papular, or urticarial type lesion
    - May precede bullae formation by months
    - Bullae small or large
    - Tense, firm topped oval or round
    - on normal, erythematous, or urticarial skin: serous / hemorrhagic; Localized / generalized; Pruritic and painful
  2. Mucosal Lesions: Erosions and ulcerations
    - Lips, oropharynx, nasal, conjunctival, vulvar, anal; Eyes- corneal ulcer, anterior uveitis
  3. Fever, weakness, malaise, and fatigue
  4. bilateral and symmetrical
23
Q

Minor EM presentation

A
  1. Little - no mucosal involvement
  2. (+) vesicle, (-) bullae
  3. no systemic symptoms
  4. confined to extremities & face
24
Q

Major EM presentation

A
  1. mucosal involvement
  2. confluence of lesions
  3. (+) Nikolsky sign
  4. (+) constitutional symptoms
  5. cheilitis/stomatitis
  6. vulvitis/balanitis
  7. eye involvement
  8. MCC - drug reaction
25
Q

mgmt for EM minor

A
  1. self limiting - remove underlying cause if applicable
  2. antihistamines for pruritus
  3. topical steroids: low dose - face/intertriginous; medium dose trunk/extremities
  4. antivirals if needed
  5. painful oral lesions:
    - high dose fluocinonide 0.05% gel 2-3x/d
    - throat Soothie/Magic Swizzle
26
Q

ingredients for throat smoothie/magic swizzle

A
  1. 1:1 of lidocaine viscous
  2. diphenhydramine (Benadryl)
  3. magnesium hydroxide (Maalox)
  4. +/- dexamethasone

1 tsp swish, gargle and spit/swallow q6h prn

27
Q

tx for EM major

A
  1. DC offending agent/tx HSV if indicated
  2. Mucocutaneous lesions
    - IV fluids if needed
    - Throat Soothie/Magic Swizzle
    - high dose PO prednisone
    - Oral/parenteral pain control
    - Wet compresses w/ Burow’s solution for large erosions
  3. Ocular involvement- Immediate referral/consult ophthalmology
28
Q

tx and complications of recurrent EM

A
  1. Daily prophylactic antiviral therapy
    - Complications: Secondary bacterial infection; Fluid/electrolyte imbalances
29
Q

Cytotoxic event caused by an immune response results in destruction of keratinocytes

A

SJS & TEN

30
Q

causes of SJS & TEN

A
  1. MC drugs
  2. Chemicals
  3. Mycoplasma
  4. Viral infections
  5. Immunization
31
Q

s/s SJS/TEN

A
  1. Fever, Chills, Malaise
  2. Headache, Sore throat, N/V/D
  3. Skin tenderness
  4. Abrupt onset of lesions
    - Macule, papule, central vesicle/bullae, erosions
    - Target lesion = 2 zones of color
    - Rapid confluence
    - +Nikolsky sign
    - Trauma - full thickness epidermal detachment: Red, oozing dermis
  5. distribution: Face and extremities - generalized
  6. Mucosal findings: Very painful, erythematous, erosions
    - Lips, Buccal mucosa, Conjunctiva, Genitalia, Anal region
  7. Eyes: Conjunctivitis, Keratitis, Synechia of the lid and conjunctiva
  8. Hair and nail: Loss of lashes and nails (TEN)
32
Q

when is SJS/TEN an emergency?

A

Systemic symptoms: Fever, HR >120 (Sloughing of epidermis)

33
Q

w/u and classification of SJS/TEN

A
  1. clinically
  2. Classification:
    - SJS = < 10% body surface
    - SJS/TEN = 10-30%
    - TEN = > 30%
  3. bx will aid
34
Q

tx for SJS/TEN

A
  1. d/c offending agent
  2. IV Fluids
  3. Parenteral nutrition
  4. IV Pain meds
  5. Wound care: Wet dressing with burow’s solution; Non adherent dressing; Eye- saline and erythromycin ointment
  6. IV Steroids/IVIG = early