Immunbullous Disease - Schwarzenberger Flashcards Preview

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Flashcards in Immunbullous Disease - Schwarzenberger Deck (30):
1

Epidemiology of Bullous pemphigoid?

Affects older people, 68-82; Women predominant

2

What is the pathophysiology of bullous pemphigoid?

Autoantibodies against the hemidesmosomes in the basal keratinocytes --> BP 180 and BP 230 proteins

3

Describe the lesions seen EARLY in bullous pemphigoid

very itchy, urticarial rash

4

Describe the blisters of bullous pemphigoid. Where are they located?

Tense (stay taut with pressure), normally symmetric in distribution. Might have erythema

5

Picture of bullous pemphigoid

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6

Mucosal involvement in bullous pemphigoid?

20% of cases; eye involvement can cause blindness, oral lesions common

7

What is gestational pemphigoid? What risks are associatesd with it?

Bullous pemphigoid arising in a pregnant woman; can cause preterm labor or low weight babies

8

What will skin biopsy of bullous pemphigoid show when immunofluoresced

IgG and C3 deposition at the dermal-epidermal junction (LINEAR PATTERN)

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9

Bullous Pemphigoid and antibodies in blood?

May or may not find antibodies in the blood

10

What is the treatment for bullous pemphigoid?

Long term systemic steroids and/or immunosuppresive Rx; requires aggressive tx

11

Epidemiology of Pemphigus vulgaris?

All ages (mean onset 50-60); male=female; incdience higher in Ashkenazi Jews and those of Mediterranean descent 

12

Pathophysiology of Pemphigus Vulgaris

Autoantibodies against the desmoglein 1 and 3

13

What is the characteristic thing seen on histology of pemphigus vulgaris?

"Row of Tombostones", intraepidermal split blisters just above the dermis 

 

 

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14

Who frequently diagnoses pemphigus vulgaris?

Dentists (LOL); commonly starts as mucosal erosions 

15

Describe the blisters seen in pemphigus vulgaris. Where are they located?

Flaccid and easily broken; commonly on the head, chest, and back 

16

Are there serum antibodies in pemphigus vulgaris?

Yes; their serum concentration (of desmoglein 1 & 3) can serve as marker for disease progression

17

What is Nikolksy sign?

When the top layers of skin slip away from the underlying layers upon light pressure

+ in Pemphigus Vulgaris (...and Stevens Johnson Syndrome/TEN)

- in Bullous Pemphigoid (tense blister, doesnt sheer laterally under pressure)

18

In Pemphigus vulgaris, where do you want to take the skin biopsy from?

The very outer edge of inflammation/erosion, adjacent to the normal skin

19

What specific drug has show great efficacy in pemphis vulgaris?

Rituximab

20

What is Dermatitis herpetiformis? 

Autoimmune bullous disease linked to celiac's and gluten hypersensitivity 

21

Dermatitis Herpetiformis: What type are the autoantibodies and what do they react with?

IgA; against tissue transglutaminase and cross react with epidermal transglutaminase

- Pt develops IgA antibodies to tissue transglutaminae (t-TG in gut)

- t-TG cross reacts with epidermal transglutaminase (e-TG in skin)

22

Where is transglutaminase found in the skin?

Dermal capillaries and the basal cells of the epidermis 

23

Epidemiology of dermatitis herpetiformis

Men>women; onsets between 30-40

24

Mucosal involvement in dermatitis herpetiformis?

NO; helps you distinguish between the other bullous diseases

25

Describe the lesions in dermatitis herpetiformis. Where are they located?

Intensely itchy rash, possibly vesicular; commonly presents on the elbows, buttocks, knees, scalp, and neck

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26

Relation between dermatitis herpetiformis and gluten sensitive enteropathy

ALL those that have dermatitis herpetiformis also have gluten sensitive disease; not vice versa

27

Histology of dermatitis herpetiformis

Lots of neutrophils, eosinophils infiltrating the dermal papillae 

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28

Treatment of dermatitis herpetiformis

Strict adherence to gluten free diet; steroids are NOT helpful

29

Dapsone used to treat which of these 3 diseases? Mechanism of action?

Treat Dermatitis herpetiformis. Prevents neutrophil infiltration into dermal papillae

30

What disease associations does dermatitis herpetiformis have? 

Non-hodgkin's lymphoma (most be worked up); other autoimmune disorders, Thyroid disease, DM, connective tissue disease