Bullous Disorders Flashcards

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1
Q

Immunobullous disorders

A

Autoimmune reactions where damage to adhesions mechanisms in the skin results in blistering

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2
Q

Bullous pemphigoid

A

IgG autoantibodies against the DEJ proteins (hemidesmosomes)

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3
Q

Blisters in bullous pemphigoid

A

Subepidermal blister: pemphidoiD = deeper blisters

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4
Q

Presentation of bullous pemphigoid

A

Large, tense blisters
Widespread
Burst to leave erosions
Healing without scarring

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5
Q

Immunofluorescence in bullous pemphigoid

A

IgG and complement at the DEJ

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6
Q

High has a higher mortality? bullous pemphigoid or bullous pemphigus

A

Bullous pemphigus

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7
Q

Bullous pemphigus

A

IgG autoantibodies against desmoglein 3 (which maintains desmosome, loss of kertinocyte adhesion)

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8
Q

Blisters in bullous pemphigus

A

Epidermal blister: pemphiguS = superficial blisters

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9
Q

What is a positive Nikolsky’s sign? What condition is it seen in?

A

The top layer of the skin slips away from the lower layers when slightly rubbed
Bullous pemphigus

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10
Q

Presentation of bullous pemphigus

A

Fluid-filled, thin roofed blisters
Burst to leave erosions
Extensive mucosal involvement may be fatal
Positive Nikolsky’s sign

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11
Q

Immunofluorescence in bullous pemphigus

A

IgG within epidermis

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12
Q

Treatment of bullous pemphigus and bullous pemphigoid

A

Steroids

Immunosuppressants

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13
Q

Dermatitis herpetiformis

A

IgA deposits in the dermal papillae causing an immunological cascade

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14
Q

Condition associated with dermatitis herpetiformis

A

Coeliac disease

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15
Q

Presentation of coeliac disease

A

Itchy lesions
Symmetrical presentation
Excoriations on elbows, knees and buttocks

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16
Q

Immunofluorescence in dermatitis herpetiformis

A

IgA in dermal papillae

17
Q

Treatment of dermatitis herpetiformis

A

Gluten free diet

Dapsone or sulphonamides (symptomatic relief)

18
Q

Epidermolysis bullosa

A

Genetic skin fragility

Defect in the proteins that attach the layers of the skin

19
Q

3 types of epidermolysis bullosa

A
Simplex = epidermis
Junctional = DEJ
Dystrophic = basement membrane
20
Q

When does epidermolysis bullosa present from?

A

From birth