First Aid, Chapter 8, Immunologic Disorders, Immunologic Skin Disease Flashcards

1
Q

What are the target skin structures for pemphigus?

A

Desmogleins-1 and -3 in desmosome for pemphigus

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2
Q

What are the target skin structures for pemphigoid and linear IgA bullous dermatosis?

A

BP Ags (BP180 and BP230) in hemidesmosome/lamina lucida for pemphigoid and linear IgA bullous dermatosis.

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3
Q

What are the target skin structures for epidermolysis bullosa acquisita?

A

Type VII collagen or anchoring fibrils for epidermolysis bullosa acquisita

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4
Q

What are the clinical signs of pemphigus?

A

Flaccid bullae on noninflamed skin Nikolsky’s sign Found on scalp, chest, oral mucosa, intertriginous areas

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5
Q

What is the target of pemphigus vulgaris?

A

IgG epithelial surface Desmoglein-3 +/– desmosome

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6
Q

What is the immunopathology of pemphigus vulgaris?

A

Epidermal IgG/C3

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7
Q

What is the treatment of pemphigus vulgaris?

A

prednisone +- immunosuppresives

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8
Q

What is the target of pemphigus folicaceous?

A

Desmoglein-1 +/– desmosome

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9
Q

What is the immunopathology of pemphigus foliaceous?

A

Epidermal IgG/C3

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10
Q

What is the treatment of pemphigus foliaceous?

A

Steroids

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11
Q

What is the target of IgA pemphigus?

A

IgA epith surface Desmocollin-1

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12
Q

What is the immunopathology of IgA pemphigus?

A

Epidermal IgA

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13
Q

What is the treatment of IgA pemphigus?

A

Steroids

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14
Q

What are the clinical findings of bullous pemphigoid?

A

Tense bullae on urticarial base
Pruritus
Flexural areas
Elderly

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15
Q

What is the target of bullous pemphigoid?

A

IgG basement membrane zone (BMZ)
BP180
BP230

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16
Q

What is the immunopathology of bullous pemphigoid?

A

IgG/C3 (BMZ)

17
Q

What is the treatment of bullous pemphigoid?

A

Steroids

Immuno-suppressives +/–

18
Q

What are the clinical signs of herpes gestationis?

A
Urticaria 
Blistering  
Periumbilical area  
During or immediately postpartum 
May flare w OCP/menses
19
Q

What is the target of herpes gestationis?

A

IgG BP180 BMZ

20
Q

What is the immunopathology of herpes gestationis?

A

Linear BMZ C3

21
Q

What is the treatment of herpes gestationis?

A

Steroids

Antihistamines

22
Q

What are the affected areas of epidermolysis bullosa acquisita?

A

Areas of trauma

Oral mucosa

23
Q

What is the target of epidermolysis bullosa acquisita?

A

Type VII collagen

24
Q

What is the immunopathology of epidermolysis bullosa acquisita?

A

Linear BMZ IgG/C3

Linear IgA/IgM

25
Q

What is the treatment of epidermolysis bullosa acquisita?

A

Colchicine or dapsone + steroids

Immuno-suppressives +/ – for refractory cases

26
Q

What are the clinical signs of dermatitis herpetiformis?

A

Small grouped vesicles LE/buttocks
Intense pruritus
Associated with celiac disease

27
Q

What is the target of dermatitis herpetiformis?

A

Endomysial IgA Transglutaminase IgA Epidermal transglutaminase

28
Q

What is the immunopathology of dermatitis herpetiformis?

A

Granular BMZ IgA

29
Q

What is the treatment of dermatitis herpetiformis?

A

Eliminate gluten from diet

30
Q

What is the mnemonic for the key features of pemphigus?

A

DEsMO (Desmosome) = DEMO.

DEMO is on the surface (of skin); so, when DEMO is affected, the surface of skin is damaged, leading to flaccid bullae.

31
Q

A 70-year-old woman presents with a pruritic rash over her upper extremities with what appear to be tense, fluid-filled blisters. What is the diagnosis?

A

Bullous pemphigoid. Tends to occur in the elderly, is pruritic, and occurs in flexural areas

32
Q

What does tissue immunofluorescence show for lupus erthematosis?

A

Granular immune deposits at BMZ in both involved and uninvolved skin (lupus band), IgM = C3 >IgG >IgA, where two or more required. Refer to Tables 8-25 and 8-29 for antigen testing in these conditions.