Vesicular/bullous Disorders

Front 1

What is the Nikolsky sign?

Back 1

1. Absence of cohesion in the epidermis

2. Upper layers are easily made to slip laterally by slight pressure or rubbing

Front 2

What is the Asboe-Hasen sign?

Back 2

1. Direct pressure on intact bulla leading to bulla-spread phenomenon

Front 3

What bx are often taken in bullous disorders?

Back 3

1. Punch

2. H&E rom edge of intact blister

3. Perilesional DIF

Front 4

What will a bx yield in pemphigus vulgaris?1.

Back 4

1. Anti-desmoglein IgG in lower epidermis

Front 5

What will a bx yield in pemphigus foliaceus?

Back 5

1. Anti-desmoglein IgG mostly in superficial epidermis

Front 6

What is desmoglein-3? Desmoglein-1?

Back 6

3--- mucosal antigen

1--- mucocutaneous antigen

Front 7

What are the ssx of pemphigus vulgaris?

Back 7

1. Flaccid bullae

2. +Nikolsky and Asboe-Hansen

3. Appear first in nose/mouth, then groin, scalp, face, neck axillae, and/or genitals

Front 8

What is the MC location of the first lesion in pemphigus vulgaris?

Back 8

1. Mouth

Front 9

What drugs can cause pemphigus vulgaris?

Back 9

1. Penicillamine

2. Captopril, other -prils

3. Piroxicam

4. Penicillin derivatives

5. Pyrazolone

Front 10

How do you tx pemphigus vulgaris?

Back 10

1. Silvadene topical

2. Maalox for mouth

3. Steroids

4. Plasmapharesis, IVIG

Front 11

What are the ssx of pemphigus vegetans?

Back 11

1. Flaccid bull that become eroded and form vegetative plaques

2. Often in body folds

3. Foul odor

Front 12

What are the ssx of pemphigus foliaceus?

Back 12

1. Flaccid bullae

2. Often only erythematous patches, crusts, or erosions--- cornflakes

3. Nikolsky sign +

4. Oral lesions rare

Front 13

What antibody is present in pemphigus foliaceus?

Back 13

1. Desmoglein 1

Front 14

What are the ssx of pemphigus erythematosus?

Back 14

1. Localized pemphigus foliaceus with SLE overlap

2. ANA+

3. Erythematous erosions, crusting

Front 15

How do you tx pemphigus erythematosus?

Back 15

1. Sun protection

2. Oral/topical steroids

Front 16

What is the MC associated underlying malignancy in paraneoplastic pemphigus?

Back 16

1. Non-hodgkin lymphoma

Front 17

What is the MC location of paraneoplastic pemphigus?

Back 17

1. Severe stomatitis extending onto vermilion lip

Front 18

What are the ssx of subcorneal pustular dermatosis?

Back 18

1. Serpiginous vesicles or pustules

2. Underlying IgA gammopathy

Front 19

What are the ssx of intraepidermal neutrophilic type IgA pemphigus?

Back 19

1. Flaccid pustules and bull involving intertriginous locations

2. Enlarge to form annular arrangement

Front 20

How do you tx both IgA pemphigus disorders?

Back 20

1. Dapsone

2. Oral corticosteroids

Front 21

What is the MC autoimmune blistering disease?

Back 21

1. Bullous pemphigoid

Front 22

What antibodies are present in bullous pemphigoid?

Back 22

1. Bullous pemphigoid antigen 1

2. Bullous pemphigoid 2

Front 23

What are the ssx of bullous pemphigoid?

Back 23

1. Urticarial wheals

2. Evolve into large, tense bull over medial thighs, groin, abdomen, and legs

3. Pruritus with subsequent tenderness

Front 24

What PMN predominates in bullous pemphigoid?

Back 24

1. Eosinophils

Front 25

How do you dx bullous pemphigoid?

Back 25

1. Linear IgG and C3 along dermal/epidermal junction

Front 26

How do you tx bullous pemphigoid?

Back 26

1. Corticosteroids

2. Tetracycline with nicotinamide TID

Front 27

What are the ssx of cicatricial pemphigoid?

Back 27

1. Scarring of mucous membrane

2. Slowly progressive shrinkage of the ocular mucous membranes and blindness

Front 28

What are the MC sites for cicatricial pemphigoid?

Back 28

1. Conjunctiva

2. Oral mucosa

Front 29

What are the antibodies associated with cicatricial pemphigoid?

Mucosa and skin?

Ocular?

Increased cancer risk?

Back 29

1. Mucosa and skin= BPAg2 antigen

2. Ocular=B4 subunit of a6-B4 integrin

3. Increased cancer= laminin 5 antigen

Front 30

What will DIF show in cicatricial pemphigoid?

Back 30

1. C3 and IgG at lamina lucida

Front 31

How do you tx cicatricial pemphigoid?

Back 31

1. Dapsone

Front 32

What is the onset of pemphigoid gestationis?

Back 32

1. 2nd or 3rd trimester

2. Postpartum

Front 33

What are the ssx of pemphigoid gestationis?

Back 33

1. Urticarial plaques and papule start around umbilicus

2. Centripetal spread

3. Tense bullae

4. Spontaneous remission

Front 34

What is the antigen involved in pemphigoid gestationis?

Back 34

1. BPAg2

Front 35

What are the risks associated with pemphigoid gestationis?

Back 35

1. Graves disease

2. Fetal prematurity

3. Small for gestational age

Front 36

What will histology show in pemphigoid gestationis?

Back 36

1. Subepidermal bulla with eosinophils

2. C3 +/- IgG at DE junction

Front 37

How do you tx pemphigoid gestationis?

Back 37

1. Oral corticosteroids

Front 38

What are the ssx of dermatitis herpetiformis?

Back 38

1. Polymorphous, grouped, symmetrical lesions

2. Often excoriated--- severe pruritus

3. Elbows, knees, buttocks, scalp, scapula

Front 39

What are the antibodies associated with dermatitis herpetiformis?

Back 39

1. Anti-endomysial

2. Anti-transglutaminase 3

3. Anti-gliadin

Formed in jejunum

Front 40

What are the risks associated with dermatitis herpetiformis?

Back 40

1. Thyroid disorders

2. Small bowel lymphoma

3. Non-Hodgkins lymphoma

Front 41

How do you tx dermatitis herpetiformis?

Back 41

1. Dapsone

2. Gluten-free diet

Front 42

What are the ssx of LABD adult form?

Back 42

1. Annular vesicles/bullae over extensor extremities and buttock

2. Oral and conjunctival lesions can scar

"Crown of jewels"

Front 43

What antibodies are associate with LABD adult and childhood forms?

Back 43

1. LAD-1

Front 44

What are the MC drug cause of LABD adult form?

Back 44

1. Vancomycin

2. Captopril, cephalosporin, penicillin

Front 45

How do you tx LBAD adult/childhood forms?

Back 45

1. Dapsone

2. Oral corticosteroid

Front 46

What will bx show in LBAD adult form?

Back 46

1. Tube of toothpaste--- IgA at basement membrane

Front 47

What are the ssx of LBAD childhood form?

Back 47

1. Bullae develop on erythematous or normal appearing skin

2. Crown of jewels arrangement

Front 48

What are the antibodies associated with EBA?

Back 48

1. Type VII collagen antibodies

Front 49

What are the ssx of EBA?

Back 49

1. Noninflammatory bullae in traumatized area

2. Skin fragility

3. Atrophic scarring

Front 50

What will a bx show in EBA?

Back 50

1. Linear IgG deposition at basement membrane

Front 51

How do you tx EBA?

Back 51

1. Steroids

2. Dapsone

3. Steroid-sparing agents

4. IVIG

Front 52

With what disorders is EBA associated?

Back 52

1. Myeloma

2. Granulomatous colitis

3. DM

4. Lymphoma

Front 53

What are the ssx of PCT?

Back 53

1. Hands and face MC involved

2. Tense bullae, erosions, milia, scarring on sun-exposed skin

3. Hypertrichosis on temples

4. Facial hyperpigmentation

Front 54

What is the MCC of PCT?

Back 54

1. Uroporphyringoen decarboxylase deficiency

Front 55

What are common triggers of PCT?

Back 55

1. Hepatitis C

2. Alcoholism

Front 56

How do you tx PCT?

Back 56

1. Phlebotomy every 2 weeks