Blistering Dermatoses Flashcards
A disease involving autoimmune destruction of desmosomes between keratinocytes…
Pemphigus Vulgaris
What is the cause of pemphigus vulgaris?
IgG antibody against desmoglein—type II hypersensitivity
What layer of the epidermis is affected in pemphigus vulgaris?
Stratum spinosum
What 2 areas of the body are affected by pemphigus vulgaris?
Skin
Oral mucosa bullae
What causes the tombstone appearance on microcopy observed with pemphigus vulgaris?
Basal layer cells attached to the basement membrane
Why do the blisters in pemphigus vulgaris rupture so easily?
Blister is suprabasal
What is seen on immunofluorescence with pemphigus vulgaris?
IgG surrounding keratinocytes in a fish-net pattern
An autoimmune destruction of the hemidesmosomes attaching the basal cells to the underlying basement membrane of the skin…
Bullous Pemphigoid
What is the cause of bullous pemphigoid?
IgG antibody against hemidesmosome components of the basement membrane
Is the oral mucosa involved with the blistering dermatosis, bullous pemphigoid?
No
What differentiates pemphigus vulgaris from bullous pemphigoid with respect to blister formation?
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.
What is seen on immunofluorescence with bullous pemphigoid?
IgG along the basement membrane in a linear pattern
An autoimmune deposition of IgA at the tips of dermal papillae…
Dermatitis herpetiformis
Dermatitis herpetiformis has a very strong association to what disorder?
Celiac disease
What is the best treatment for dermatitis herpetiformis?
Gluten-free diet
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?
Erythema Multiforme
What type of reaction is involved with erythema multiforme?
Hypersensitivity reaction
What is the most common infection association with erythema multiforme?
Herpes simplex virus infection
Besides the most common association with erythema multiforme, HSV infection, what are some other associations seen with erythema mulltiforme?
Mycoplasma infection
Drugs (penicillin and sulfonamides)
Autoimmune disease (SLE)
Malignancy
What syndrome is used to describe erythema multiforme with oral mucosa/lip involvement and a fever?
Stevens-Johnson syndrome
What is toxic epidermal necrolysis with respect to erythema multiforme and Stevens-Johnson syndrome?
TEN is a severe form of SJS and is characterized by sloughing of skin (resembles a burn), usually caused by an adverse drug reaction
