Blistering Dermatoses

Question 1

A disease involving autoimmune destruction of desmosomes between keratinocytes…

Answer 1

Pemphigus Vulgaris

Question 2

What is the cause of pemphigus vulgaris?

Answer 2

IgG antibody against desmoglein—type II hypersensitivity

Question 3

What layer of the epidermis is affected in pemphigus vulgaris?

Answer 3

Stratum spinosum

Question 4

What 2 areas of the body are affected by pemphigus vulgaris?

Answer 4

Skin

Oral mucosa bullae

Question 5

What causes the tombstone appearance on microcopy observed with pemphigus vulgaris?

Answer 5

Basal layer cells attached to the basement membrane

Question 6

Why do the blisters in pemphigus vulgaris rupture so easily?

Answer 6

Blister is suprabasal

Question 7

What is seen on immunofluorescence with pemphigus vulgaris?

Answer 7

IgG surrounding keratinocytes in a fish-net pattern

Question 8

An autoimmune destruction of the hemidesmosomes attaching the basal cells to the underlying basement membrane of the skin…

Answer 8

Bullous Pemphigoid

Question 9

What is the cause of bullous pemphigoid?

Answer 9

IgG antibody against hemidesmosome components of the basement membrane

Question 10

Is the oral mucosa involved with the blistering dermatosis, bullous pemphigoid?

Answer 10

No

Question 11

What differentiates pemphigus vulgaris from bullous pemphigoid with respect to blister formation?

Answer 11

Bullous pemphigoid blisters are much thick due to the detachment of the basal cells from the basement membrane causing a subbasal blister, which do not rupture as easy.

Question 12

What is seen on immunofluorescence with bullous pemphigoid?

Answer 12

IgG along the basement membrane in a linear pattern

Question 13

An autoimmune deposition of IgA at the tips of dermal papillae…

Answer 13

Dermatitis herpetiformis

Question 14

Dermatitis herpetiformis has a very strong association to what disorder?

Answer 14

Celiac disease

Question 15

What is the best treatment for dermatitis herpetiformis?

Answer 15

Gluten-free diet

Question 16

A patient presents with a targetoid rash and blisters along the dorsal surface of his hand. The patient has a recent history of HSV infection. What is the most likely diagnosis?

Answer 16

Erythema Multiforme

Question 17

What type of reaction is involved with erythema multiforme?

Answer 17

Hypersensitivity reaction

Question 18

What is the most common infection association with erythema multiforme?

Answer 18

Herpes simplex virus infection

Question 19

Besides the most common association with erythema multiforme, HSV infection, what are some other associations seen with erythema mulltiforme?

Answer 19

Mycoplasma infection
Drugs (penicillin and sulfonamides)
Autoimmune disease (SLE)
Malignancy

Question 20

What syndrome is used to describe erythema multiforme with oral mucosa/lip involvement and a fever?

Answer 20

Stevens-Johnson syndrome

Question 21

What is toxic epidermal necrolysis with respect to erythema multiforme and Stevens-Johnson syndrome?

Answer 21

TEN is a severe form of SJS and is characterized by sloughing of skin (resembles a burn), usually caused by an adverse drug reaction