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Dermatology Pathology > Immunobullous Disease > Flashcards

Immunobullous Disease Flashcards

1
Q

What is the general causes of vesicles in a straight line?

A

Most often this is an exogenous cause like poison ivy etc. because the body doesn’t make straight lines

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2
Q

What is bullous diabeticoum?

A

Blisters arising on the palm from diabetes

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3
Q

What is a coma blister?

A

Blister that arises from long standing pressure, often associated with barbituate overdose

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4
Q

What is this?

A

Bullous Impetigo

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5
Q

What is this?

A

Edema blister - notice the redness and and inflammation in all the tissue surrouding the blister.

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6
Q

These non-blanching lesion were found on a patient. Is it possible that these are morbilliform lesions?

A

NO, morbilliform lesions blanch. This is a vasculitis.

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7
Q

What is this?

A

Bullous fixed drug reaction, notice the alteration of pigmentation around the blister

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8
Q

This patient has leukemia. What could this be?

A

Exuberant bug bite in a patient with leukemia

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9
Q

What is a key feature to distinguish bed bug bites from other types of bug bites?

A

Groups of 3 = bed bug bites, breakfast, lunch, and dinner

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10
Q

Cutaneous Mastocytoma - occurs in kids with mastocytosis

A
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11
Q

What is this?

A

Epidermolysis Bullosa - this kid was born with this

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12
Q

Autoimmune bullous diseases
• who do we usually see with this?
• what are some non-ideopathic causes?

A

Autoimmune bullous diseases typically occur in older people and is most often ideopathic, but may be associated with neoplasms.

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13
Q

Who shoud you suspect autoimmune blistering diseases in?

A

Suspect in patients with unexplained blisters, erosions, erosions or pain of mucous membrane, itchy rash that doesn’t respond to typical treatments.

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14
Q

What autoimmune blistering diseases do we need to know?
• which are most common?

A

Most Common:
Bullous Pemphigoid and Variants
Pemphigus Vulgaris and Variants
Dermatitis herpetiformis

Less Common:
Epidermolysis bullosa acquisita
Linear IgA bullous dermatosis
IgA pemphigus
Bullous systemic lupus erythematsus

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15
Q

What drug should you think of when you think of epidermolysis bullosa acquisita?

A

Vancomycin

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16
Q

Normal Skin Histology

A
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17
Q

What do desmisomes connect?
• hemidesmisomes?

A

Desmisomes connect one keritinocyte to the other

Hemidesmosomes connect the basal cells to the basement membrane

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18
Q

What autoimmune bullous disease is this?
How do you know?
What do you expect to see on histology?

A

Bullous Pemphigoid - differentiate from vulgaris on the basis of in tact vesicles that aren’t typically seen in pemphigus vulgaris

Histology should show the epidermis pulling away from the dermis (split at the EDJunction). Also eosinophilia is also common at the EDJ that is falling off.

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19
Q

What autoimmune disease is this?
• How will it present at onset?
• how will it progress?
• Mucous membrane involvment?

A

Bullous Pemphigoid often starts as a very itchy urticarial rash in a WOMAN between 68-82 or older that doesn’t resolve. It will then progress into tense blisters that are typically symetrically distributed ± erythema. Mucous membranes involved less than 20% of the time. have no known etiology or sometimes they’re paraneoplastic.

**Note while a small portion of these patients get mucous membrane involvment, I think SJS should be assumed with most patients with SJS**

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20
Q

What are the fluorescent antibodies in this picture targeting?

A

The bright layer is due to the staining of hemidesmosomes on IF the target antigens are BP180 and BP230

Bullous Pemphigoid 180 and 230.

21
Q

Is this SJS or Bullous Pemphigoid?
• how do you know?

A

While you see split at the EDJ in both disorders, there is eosinophilia here that is indicative of BP not SJS

**Below is a zoomed out picture**

22
Q

This person has had an itchy rash for weeks now and no etiology can be determined. What would probably be a reasonable next step to diagnose this patient?

A

He looks older so may fall in that 62-82 age bracket for BP. Do a biopsy and look for a band of IgG and C3 at the EDJ. Antibodies may or may not be present in the blood.

23
Q

BP - HOW do you treat this disease?
• how persistent is it?

A

BP - treated with systemic steroids ± immunosuppressives

BP often lasts for several years then remits

24
Q

BP

25
Wha two variants of bullous pemphigoid should you be aware of?
Mucous membrane pemphigoid Gestational Pemphigoid "herpes gestationis"
26
What are the characteristics or mucous membrane pemphigoid?
* Scarring eye involvment that can lead to blindness * oral lesions * ± skin lesions
27
Gestational Pemphigoid "Herpes gestationis" • When does it happen? • Possible pregnancy outcomes associated with it? • Persistence?
**Gestational Pemphigoid** typcially arises in the **2nd trimester** of pregnancy and is often associated with **preterm labor** or **low weight-for-gestational age** babies. This is **NOT a persistent disease, it remits post-pardum**. BUT often **comes back with additional pregnancies.**
28
What disease is this? • how do you know? • what would you expect to see on gross inspection?
This is pemphigus vulgaris - you can tell because the **EDJ is in tact** but the **stratum spinosum** is pulled off of the basal layer. This leaves behing **tombstone** basal cell. • Grossly you expect to see superficial blisters that are not in tact but instead are eroded - see below
29
Pemphigus vulgaris is less common than bullous pemphigoid? • who does it typically affect? • Cause?
Unlike bullous pemphigoid (which affects mostly women), **pemphigus vulgaris** affects **men and women equally** with a **younger age of onset starting in the 50's and 60's** most often. Pemphigus vulgaris may have a **genetic predisposition.**
30
The target antigens in bullous pemphigoid are 1 and 3. What are the target antigens of pemphigus vulgaris?
**Desmoglein 1 and 3** are the target antigens in pemphigus vulgaris
31
What is the name for loss of cell-cell attachment seen in pemphigus vulgaris? • what do you expect to see on immunofluorescence?
**ACANTHOLYSIS** IF: shows a **NETLIKE** pattern with **IgG and C3** that outline the desmogliens 1 and 3.
32
What key tissue is involved in pemphigus vulgaris that may help to differentiate it from bullous pemphigoid?
PV often involves painful erosion of the mouth and gums - remember that BP only involves mucous membranes in 1/5 cases
33
What do you expect to see on inspection of someone with pemphigus vulgaris?
Blisters *may or may not* be present on the skin, but if they're there they are typically **flaccid and easily broken.** The most common places to see blisters are on the **head, trunk, and flexural surfaces**. IMPORTANTLY **mucous membrane involvment may be extensive and the erosions are painful.**
34
What mucosa can be involved in pemphigus vulgaris?
ALL of it, larynx, esophagus, conjunctiva etc.
35
This may resemble SJS, but what could clue you in that its not?
Scarring tells you its probably not SJS, lack of blistering tells you its probably not Bullous pemphigoid
36
PV
37
How do you diagnose pemphigus vulgaris? • How can you track the progress? • how do you treat it?
Pemphigus Vulgaris is diagnosed with a skin biopsy that shows tombstone basal cells with a reticular pattern on IF. You can track PV using serum antibodies (note that this is not the case with bullous pemphigoid) TX: **long term steriods and immunosuppressives** if you don't treat this disorder it is **FATAL 70% of the time.**
38
What two variants of pemphigus vulgaris do you need to know?
Pemphigus Foliaceus Paraneoplastic Pemphigus
39
Pemphigus foliaceus • what is it? • where is it common?
Pemphigus foliaceus is **common in Brazil** and is the formation of **anti-desmoglein 1** antibodies that causes a **superficial pemphigus** causes a corn flake-like appearance.
40
What is paraneoplastic pemphigus?
Pemphigus associated with **m**alignancy that involves intense **m**ucosal damage.
41
Dermatitis herpetiformis • what is the pathophysiology behind this disease? • what diseas is very similar?
IgA antibodies form against **EPIDERMAL transglutamase** that cross reacts with reticulin fibers that attach the **PAPILLAE to the basement membrane.** **Celiac disease** is very similar in that IgA antibodies are directed against **TISSUE transgluaminase**
42
What is the relationship between celiac disease and dermatitis herpatiformis?
ALL patients with dermatitis herpatiformis are assumed to have celiac disease (or just general enteropathy). HOWEVER, patients with celiac disease do not necessarily have dermatitis herpatiformis
43
Who typically presents with dermtitis herpatiformis?
This is typically a **male** in his **30's to 40's** that presents with **GI SYMPTOMS** (cramping and diarrhea). His skin rash may be **crazy itchy** but *doesn't always have **vesicles**.* Most often this rash is **SYMMETRIC** on the **Elbows, butt, knees, scalp, and neck.**
44
How common is mucous membrane involvment in Dermatitis herpetiformis?
Not common at all.
45
What do you expect histology to look like for this disease? • what is the key to this IF?
IF: • **GRANULAR IgA** and **C3 deposition** at dermal papillae is key to knowing **dermatitis herpetiformis** **Histology: Neutrophilic, eosinophilic inflitrate at the dermal papillae**
46
Do you expect the vesicle in Dermatitis herpetiformis to be pyogenic?
Yes, the infiltrate at the **papillae** is **neutrophilic** making is a mini-**abscess**
47
How do you treat Dermatitis Herpetiformis?
* *• STOP EATING GLUTEN** - will control both GI and Skin symptoms * *• DAPSONE** - controls skin itch, but doesn't do anything for GI
48
What diseases increase you chances of getting Dermaititis Herpetiformis? • what does DH increase your risk of getting?
DH associated with: • Autoimmune conditions - thyroid dz and CT diseases DH pts. at increased risk of lymphoma
49

Dermatology Pathology (10 decks)

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