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MSK II Final > Matthys: Vesiculobullous Diseases > Flashcards

Matthys: Vesiculobullous Diseases Flashcards

1
Q

Vesicle

A

-a blitster less than 1 cm in size

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2
Q

Bulla

A

bilster greater than 1 cm

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3
Q

Vesiculobullous

A

-blisters between > and < 1 cm

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4
Q

Impetigo

A
  • highly contagious gram + bacterial infection of the superficial layers of the skin
  • Staph Aureus group 2 phage 71?GABHS
  • Bullous/nonbullous
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5
Q

Path of impetigo

A
  • Group 2 S. Aureus
  • nares small percentage groin, hand, rectum, pharynx
  • normal flora
  • exfoliative endotoxins A nd B
  • neonates and infants most common for bullous
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6
Q

Clinical presentation of impetigo

A
  • rapid blister, asymptomatic
  • hot weather
  • crowded condition
  • poor immune function
  • atopic dermatitis, herpe infection, trauma
  • flaccid <1 cm bulla, usuallly ruptured
  • collarete of scale
  • no sore throat or LAD
  • face most common ,but anywhere on body
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7
Q

ddx for impetigo

A
  • Herpes simplex
  • tinea pedis
  • bullous pemphigoid
  • staph scalded skin
  • pemphigus vulgaris
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8
Q

Tx for impetigo

A

-abx

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9
Q

Staphylococcal scalded skin syndrome

A
  • toxin mediated exfoliative dermatitis
  • severe impetigo to widespread skin denudation
  • exfoliation followed by painful erythema
  • infants and young children
  • nurseries and daycare
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10
Q

pathophys of scalded skin syndrome

A
  • S aureus type 2
  • exotoxine 1 and 2
  • blister at the granular layer of the epidermis splitting the desmosomes
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11
Q

clinical presentation of scalded skin sydnrome

A
  • focus of infection
  • fever, faint orange red macules
    • nikolsky sign
  • paper like wrinklying of the peidermis
  • cultures are sterile
  • dramatic….. heals in 5-7 days
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12
Q

what is nikolsky sign?

A

-dislodgement of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin

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13
Q

ddx for scalded skin syndrome

A
  • scarlet fever
  • kawasaki disease
  • TEN
  • EM
  • child abuse
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14
Q

tx of scalded skin syndrome

A
  • abx
  • fluids
  • make the diagnosis
  • culture/bx….. frozen section
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15
Q

Pemphigus vulgaris

A
  • Autoimmune blistering disease of the skin and oral mucosa

- circulating IgG antibody against the intraepidermal keratinocytes

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16
Q

pathophys of pemphigu vulgaris

A
  • Binding of IgG autoantibodies causes loss of adhesion between keratinocytes
  • Desmoglein 3
  • circulating IgG1 and 4
  • disease activity correlates with circulating antibodies
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17
Q

clinical presentation of pemphigus vulgaris

A
  • all races
  • 50-70% oral mucosa involvemment
  • flaccid bulla on the skin with an erosion in the oral mucosa
  • +nikolsky sign
  • +absoe-Hansen sign- lateral pressure on the bulla will spread the bulla to uninvolved skin
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18
Q

Causes of pemphigus vulgaris

A
  • genetic
  • age
  • disease association…. myasthenia gravis and thymoma
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19
Q

ddx of pemphigu vulgaris

A
  • Bullou spemphigoid
  • Hailey-Hailey disease
  • linear IgA disease
  • Pemphigus Foliaceus
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20
Q

dx and tx of pemphigus vulgaris

A
  • biopsy
  • IDIF
  • Prednisone, Imuran
  • Ophthamologist
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21
Q

Herpes Simplex

A
  • 1 is above belt, 2 is below
  • 1 causes encephalitis 60-80% mortality
  • Primary infection, latency, and recurrence
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22
Q

pathophys of HSV

A
  • 1= respirator droplets
  • 2= genital contact
  • cytolytic infection with fluid filled vesicles
  • 70-90% of population has HSV1 vs 22% has HSV2
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23
Q

clinical presentation of HSV

A
  • asymptomatic to painful; prodrome of itching and burning
  • herpetic whitlow andherpes gladiatorum
  • recurrent lesions common
  • immune status
  • encephalitis
  • Vesicular or ulcerative on an erythematous base
  • oral, genital, body, keratoconjunctival, encephalitis, anal
  • Seizures, headache
24
Q

ddx of HSV

A
  • Chancroid
  • Urethritis
  • HAnd foot and mouth disease
  • Proctitis
25
Q

dx and tx of hsv

A
  • culture or biopsy (tzanck)

- antivirials

26
Q

Chicken pox

A

-self limiting, contagious, viral infection
-4 million children per year
-4000 million in lost wages per year
=- 50-100 deaths annually in US

27
Q

pathophys of chicken pox

A
  • primary varicella virus; herpes family type 3
  • respirator and direct contact with skin
  • 10-21 day incubation
  • Infectious 1-2 days prior to the rash and until the lesions crust
28
Q

clinical presentation of chicken pox

A
  • hx of recent outbreaks
  • itchy rash
  • “oval teardrop on an erythematous base”
  • “dew drop on a rose pedal”
  • face and trunk
  • All stages
  • fever, shallow aphthous ulcers on oral mucosa
  • spares the distal extremity
29
Q

ddx of chicken pox

A
  • impetigo
  • herpes simplex
  • herpes zoster
30
Q

dx and tx of chicken pox

A
  • no ASA
  • VZIG
  • Antivirals
  • Antihistamines
31
Q

Bullous pemphigoid

A
  • chronic, AI, subepidermal blistering disease that RARELY involves the oral mucosa
  • Autoantibodies of IgG against the BP antigens 230 and 180 (BP Ag1 and 2)
  • uncommon
32
Q

Pathophys of Bullous pemphigoid

A
  • IgG bind to skin atibodies activation complement and inflammatory mediators
  • Inflammatory cells release proteases causeing bulla formation
  • Serum levels of BP Ag2 may correlate with disease activities in some pts
33
Q

clinical presentation of Bullou spemphigoid

A
  • subactute or acute formation of tense bulla
  • UV radiation or drug induced
  • Bullous, vesicular, urticarial , acral in children
  • Causes include genetic, age, epitope spreading
34
Q

ddx for bullous pemphigoid

A
  • cicatrical pemphigoid
  • drug induced pemphigoid
  • Herpes Gestationis
35
Q

dx ant x of bullous pemphigoid

A
  • Biopsy
  • IDIF (indirect immunofluoresence?)
  • Prednisone and other immunosuppresives
36
Q

Polymorphous light eruption

A
  • idiopathic photodermatoses relating to sun exposure
  • papules, vesicles, and plaques
  • 10-21% of all people
  • 75% American Indian
  • happens more with women
37
Q

pathophys of PLE

A
  • unknown
  • type IV delayed hypersensitivity rxn
  • UV-A induced ICAM-1
38
Q

Clinical presentation of PLE

A
  • sunlight is etiologic factor most of time
  • spring, lessens throughout summer
  • about 30 minutes after sun exposure
  • papules, vesicles, urticaria, or EM type features
  • UV-A or UV-B
39
Q

ddx for PLE

A
  • subacute lupus
  • contact dermatitis
  • solar urticaria
40
Q

dx and tx for PLE

A
  • R/O porphyria, lupus
  • photopatch testing
  • photo testing MED
  • Topical steroid, oral or IM steroids, antimalarials
41
Q

Dermatitis Herpetiformis

A
  • Immune mediated skin disease with a gluten sensitive enteropathy
  • rare in african american
42
Q

pathophys of DH

A
  • skin and GI affected
  • circulating immune complexes are found
  • IgA binds antigen in bowel and skin
  • Increased expression of HLA-B1, B8, DR8, and DRQ
  • Patient have uniform gluten sensitivity
43
Q

clinical presentation of DH

A
  • pruritic, burning skin blisters distributed in a symmetric fashion; grouped
  • Lasts days to weeks
  • Urticarial wheal to vesicle to bulla
44
Q

ddx for DH

A
  • Allergic contact dermatitis
  • Atopic Dermatitis
  • Scabies
  • Neurotic excoriations
  • Papular Urticaria
  • Bullous pemphigoid
45
Q

dx and tx for DH

A
  • HE and DIF biopsy
  • Clinical exam
  • Be aware of associated diseases
  • Bapsone and gluten free diet
46
Q

Erythema Multiforme

A
  • Benign self limited eruption classically seen as targetoid or iris shaped macules or vesiculopapules on the palms or soles
  • Minor/major
  • 50% under age 20
  • seems relatively rare
47
Q

pathophys of EM

A
  • not understood/HSV cell mediated response
  • Infection major cause
  • Drugs….. sulfa/phenytoin/ PCN/allopurinol
  • Foods, sunlight, contactants, etc
48
Q

clinical presentation of EM

A
  • dull red macules or papules with vesicles on palms or soles with little mucosal involvement
  • Iris or target like
  • bilateral and often symmetric
  • polycyclic or arcuate
  • Koebner Phenomenon
49
Q

where will EM present on the body

A
  • mucosal involvement 70%
  • lips
  • anus
  • eyes
  • oropharynx
50
Q

ddx for EM

A
  • drugs
  • syphillis
  • psoriasis
  • BP
  • SSSS
  • Kawasaki’s disease
51
Q

dx and tx for EM

A
  • biopsy
  • find cause
  • antivirals
  • Steroids?
52
Q

Toxic Epidermal necrolysis

A
  • extensive full thickness skin detachment
  • EM spectrum
  • Early sx non specific
53
Q

Pathophysiology of TEN

A
  • HLA B12
  • SLE
  • HIV
  • Medicines, infection: cell mediated cytotoxic rxn against epidermal cells, CD8 cells, macs, cytokines
54
Q

clinical presentation of TEN

A
  • 1-3 week proderome
  • fever, skin tenderness
  • skin pain, burning
  • Newly added drugs most common
55
Q

Clinical presentation of TEN

A
  • Morbilliform, EM type eruption
  • Skin with crinkled surface
  • sheet like epidermal loss
  • +nikolsky sign
  • Mucosal membrane involvement
56
Q

ddx for TEN

A
  • EM
  • Scarlet fever
  • GVHD
  • SSSS
  • Exfoliative dermatitis
  • Genreralized bullous fixed drug eruption
57
Q

dx and tx for TEN

A
  • D/C drug suspected
  • IV fluids
  • Burn unit
  • Steroids as a last resort
  • IV Ig
  • Prevent infection

MSK II Final (13 decks)

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