Vesiculobllous Diseases Flashcards

1
Q

What is the specific pattern of IgG binding immunohistochemistry of pemphigus vulgaris

A

Fishnet pattern of binding

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2
Q

In pemphigus vulgaris there is acantholysis that form rounded cells. what are they called? 

A

Tzanck cells (they have enlarged hyperchromatic nuclei)

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3
Q

In pemphigus vulgaris there is abnormal production of auto antibodies, which ones are they?

A

Desmoglein 1 and Desmoglein 3

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4
Q

Which drugs can cause pemphigus

A
  1. Captopril
  2. rifampin
  3. penicillin
  4. phenobarbital
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5
Q

Which Neoplasms are associated with paraneoplastic pemphigus

A
  1. Non-Hodgkin lymphoma
  2. chronic lymphocytic leukaemia
  3. Castleman disease
  4. thymoma
  5. spindle cell tumours
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6
Q

What is Behçet’s disease

A

Characterized by recurrent aphthous stomatitis and at least two of the following:
1. genital ulcers
2. Eye lesions
3. Skin lesions
4. Rapid acute inflammation of skin in response to minor trauma

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7
Q

Which type of pemphigoid shows positive for indirect immunoflouresence

A

Bullous pemphigoid

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8
Q

Which type of pemphigoid how to split between the surface epithelium in the underlying connective tissue as well as vasodilation

A

Mucous membranes pemphigoid

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9
Q

What type of circulating auto antibodies are produced in mucous membrane pemphigoid

A

Epiligrin (laminin - 5)

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10
Q

What is symblepharons? Which pemphigoid causes it?

A

Scaring between bulbar and palpebral conjunctivae which lead to adhesion of both. Leads to blindness. In mucous membrane pemphigoid

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11
Q

name the types of epidermolysis bullosa

A
  1. Epidermolysis bullosa simplex
  2. Junctional epidermolysis bullosa
  3. Dystrophic
  4. Acquired acquista
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12
Q

Which genes are mutated in epidermolysis bullosa simplex

A

Keratin 5
Keratin 14

Structure: linking proteins

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13
Q

Which genes are mutated in junctional epidermolysis bullosa

A

Lamanin - 332
Type 17 collagen
Alpha 6 beta 4 integrin

Structure - anchoring filaments

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14
Q

Which genes are mutated in dystrophic (epidermolysis bullosa)

A

Type 7 collagen

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15
Q

Which genes are mutated in acquired acquisita (epidermolysis bullosa)

A

Type 7 collagen

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16
Q

what are the dentAl manifestations of epidemolysis bullosa

A
  1. Enamal hypoplasia
  2. Rapid Carie’s development
  3. Ankyloglossia
  4. Gingival erythema
  5. Gingival recession
17
Q

Acquired acquisita ( epidemilysis bullosa ) is associated with which diseases

A
  1. Multiple myeloma
  2. DM
  3. Amyloidosis
  4. TB
  5. IBS
18
Q

What factors precipitate erythema multiforme

A
  1. Infections
  2. Drugs
  3. GI conditions
  4. Malignancies
  5. Radiation therapy
  6. Vaccination
19
Q

Write the histo pathology of erythema multiform

A
  1. Intracellular and intercellular edema
  2. Pooling of eosinophilia amorphous coagulam within the epithelium
  3. Migration of mononuclear and poly nuclear cells into layers of epithelium
  4. Vasodilation
  5. Acanthosis and elongation of rete pegs
  6. Interstitial transudate pooling
  7. Perivascular infiltrate around blood vessels
20
Q

Immunoflouresence of erythema multiform shows what?

A

IgM
C3

21
Q

What are the types of erythema multiform major

A
  1. Stevens Johnson syndrome
  2. toxic epidermal necrolysis 
22
Q

Which type of drugs should be avoided in toxic epidermal necrolysis 

A

Corticosteroids

23
Q

What are the three types of recurrent aphthous ulcerations

A
  1. Aphthous minor
  2. Aphthous major
  3. Herpetiform ulcers
24
Q

What are the causes of recurrent aphthous ulcerations

A
  1. Crohn’s disease
  2. Gluten sensitive enteropathy (Celiac disease)
  3. Deficiency (iron, folic acid, B12)
  4. Certain food ingestion
  5. Menstrual cycle
  6. Chronic asthama, allergies
  7. Anxiety and stress
  8. HIV
25
Q

What is the pathergy test and what is it used for?

A

Abnormal intense inflammatory reaction to an intradermal injection of a small amount of saline.

Used to test for behçets syndrome