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
dx and tx of hsv
- culture or biopsy (tzanck) | - antivirials
26
Chicken pox
-self limiting, contagious, viral infection -4 million children per year -4000 million in lost wages per year =- 50-100 deaths annually in US
27
pathophys of chicken pox
- 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
clinical presentation of chicken pox
- 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
ddx of chicken pox
- impetigo - herpes simplex - herpes zoster
30
dx and tx of chicken pox
- no ASA - VZIG - Antivirals - Antihistamines
31
Bullous pemphigoid
- 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
Pathophys of Bullous pemphigoid
- 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
clinical presentation of Bullou spemphigoid
- 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
ddx for bullous pemphigoid
- cicatrical pemphigoid - drug induced pemphigoid - Herpes Gestationis
35
dx ant x of bullous pemphigoid
- Biopsy - IDIF (indirect immunofluoresence?) - Prednisone and other immunosuppresives
36
Polymorphous light eruption
- idiopathic photodermatoses relating to sun exposure - papules, vesicles, and plaques - 10-21% of all people - 75% American Indian - happens more with women
37
pathophys of PLE
- unknown - type IV delayed hypersensitivity rxn - UV-A induced ICAM-1
38
Clinical presentation of PLE
- 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
ddx for PLE
- subacute lupus - contact dermatitis - solar urticaria
40
dx and tx for PLE
- R/O porphyria, lupus - photopatch testing - photo testing MED - Topical steroid, oral or IM steroids, antimalarials
41
Dermatitis Herpetiformis
- Immune mediated skin disease with a gluten sensitive enteropathy - rare in african american
42
pathophys of DH
- 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
clinical presentation of DH
- pruritic, burning skin blisters distributed in a symmetric fashion; grouped - Lasts days to weeks - Urticarial wheal to vesicle to bulla
44
ddx for DH
- Allergic contact dermatitis - Atopic Dermatitis - Scabies - Neurotic excoriations - Papular Urticaria - Bullous pemphigoid
45
dx and tx for DH
- HE and DIF biopsy - Clinical exam - Be aware of associated diseases - Bapsone and gluten free diet
46
Erythema Multiforme
- 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
pathophys of EM
- not understood/HSV cell mediated response - Infection major cause - Drugs..... sulfa/phenytoin/ PCN/allopurinol - Foods, sunlight, contactants, etc
48
clinical presentation of EM
- 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
where will EM present on the body
- mucosal involvement 70% - lips - anus - eyes - oropharynx
50
ddx for EM
- drugs - syphillis - psoriasis - BP - SSSS - Kawasaki's disease
51
dx and tx for EM
- biopsy - find cause - antivirals - Steroids?
52
Toxic Epidermal necrolysis
- extensive full thickness skin detachment - EM spectrum - Early sx non specific
53
Pathophysiology of TEN
- HLA B12 - SLE - HIV - Medicines, infection: cell mediated cytotoxic rxn against epidermal cells, CD8 cells, macs, cytokines
54
clinical presentation of TEN
- 1-3 week proderome - fever, skin tenderness - skin pain, burning - Newly added drugs most common
55
Clinical presentation of TEN
- Morbilliform, EM type eruption - Skin with crinkled surface - sheet like epidermal loss - +nikolsky sign - Mucosal membrane involvement
56
ddx for TEN
- EM - Scarlet fever - GVHD - SSSS - Exfoliative dermatitis - Genreralized bullous fixed drug eruption
57
dx and tx for TEN
- D/C drug suspected - IV fluids - Burn unit - Steroids as a last resort - IV Ig - Prevent infection