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B1-2 Vesiculobullous Diseases
N/A
38
Medical
Graduate
09/05/2012

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Term
What is vesiculobullous disease?
Definition
Disease state characterized by persistent formation of either vesicles or bullae
Term
What are the major autoimmune blistering diseases?
Definition
Pemphigus, bullous pemphigoid, linear IgA bullous dermatoses, Dermatitis Herpetiformis
Term
What causes autoimmune blistering diseases?
Definition
Production of auto-antibodies that attack a specific adhesion protein in the skin that form either desmosomes or hemidesmosomes causing the cells of the epidermis or the cells holding the epidermis to the dermis to become "unglued"
Term
What causes a blister?
Definition
When skin cells become "unglued." Location depends on the adhesion proteins involved. Desmosomes = epidermis, Hemidesmosomes = subepidermal. Location correlates with type of disease
Term
Pemphigus is what type of autoimmune blistering disease?
Definition
Intraepidermal, PV is above basal layer of epidermis, PF is below corneal layer of epidermis
Term
Bullous Pemphigoid is what type of autoimmune blistering disease?
Definition
Subepidermal
Term
What is a flaccid blister?
Definition
Fragile blister that is easily ruptured, indicates intraepidermal blister that is higher up
Term
What is a tense blister?
Definition
Firm blister not easily ruptured, indicates deeper subepidermal blister
Term
What is a Nikolsky sign?
Definition
Pressing lightly on the edge of the blister causes superficial skin to tear away, indicates fragile blister, seen in Phemphigus but not BP
Term
What is a Asboe-Hanson sign?
Definition
Pressing lightly on the blister causes fluid to spread into surrounding intact skin, seen in Phemphigus but not BP
Term
How are blisters biopsied?
Definition
Punch biopsy and then examination under light microscope with H&E staining to determine which layer is affected, then sent for direct immunofluourescence
Term
What are the different presentations of disease under direct immunofluorescence?
Definition
Dermatitis Herpetiformis = granular deposition of IgA, Linear IgA = linear deposition of IgA, bullous pemphigoid = linear deposition of IgG
Term
What are the characteristics of Pemphigus Vulgaris
Definition
Common form, autoimmune blistering disease that can affect both skin and mucous membrane, has 2 types - mucosal dominant (mucosal only) and mucocutaneous (mucosal + skin), can be life threatening
Term
How does Pemphigus Vulgaris present?
Definition
All patients have oral mucosal lesions which precede skin lesions, characteristically flaccid blisters in upper epidermis, heals with brown hyperpigmentation w/out scarring
Term
What auto-antibodies are involved in Pemphigus Vulgaris?
Definition
Desmoglein 3 only in mucosal dominant, Desmoglein 1 and 3 in mucocutaneous
Term
How does Pemphigus Vulgaris present histologically?
Definition
Intraepidermal blister w/ suprabasilar acantholysis and blister with
“tombstoning” of the basal layer
Term
How is Pemphigus Vulgaris treated?
Definition
Aimed at stopping the production of autoantibodies via corticosteroids and immunosuppressants
Term
What is the clinical presentation of Pemphigus Foliaceous?
Definition
Cutaneous involvement without mucosal lesions, small, flaccid blisters that
are superficial, fragile and easily rupture, more superficial than Pemphigus Vulgaris, crusting may resemble corn flakes, Nikolsky sign positive
Term
What auto-antibodies are involved in Pemphigus Foliaceous?
Definition
Desmoglein 1 only
Term
How do you differentiate Pemphigus Foliaceous from Pemphigus Vulgaris?
Definition
In Pemphigus Foliaceous, blister occurs higher up epidermis, desmoglobin 1 only, does not involve mucous membrane
Term
What is the most common type of autoimmune blistering disease?
Definition
Bullous Pemphoid, causes subepidermal blistering and most commonly occurs in the elderly
Term
How does Bullous Pemphoid present clinically?
Definition
Widespread TENSE blisters w/good structural integrity, can be very pruritic, negative Nikolsky sign
Term
What medications are recommended for treating Bullous Pemphigoid?
Definition
Lasix (furosemide) and Sulfa based drugs such as captopril, penicillamine, and gold
Term
What auto-antibodies are involved in Bullous Pemphigoid?
Definition
Directed against components of hemidesmosomes, specifically BP Ag1 (BP230) and BP Ag2 (BP180)
Term
How is Bullous Pemphigoid treated?
Definition
orticosteroids and immunosuppressants aimed at decreasing antibody response
Term
How does Bullous Pemphigoid present histologically?
Definition
Subepidermal blister w/numerous eosinophils, Ab's (IgG or complement C3) fluoresce in a linear "roof" pattern upon direct immunofluourescence
Term
What are the characteristics of Linear IgA Bullous Dermatosis?
Definition
Autoimmune subepidermal blistering disorder w/vesicles and bullae (some are annular), lesions are variable presentation
Term
What causes Linear IgA Bullous Dermatosis?
Definition
Often drug induced in adults
Term
How is Linear IgA Bullous Dermatosis treated?
Definition
Dapsone, Sulfapyridine, Oral Corticosteroids
Term
What are the characteristics of Dermatitis Herpetiformis?
Definition
AKA Duhring's disease, cutaneous manifestation of celiac disease, associated with gluten insensitivity, onset between ages 20-40, severely pruritic lesions, common on extensors, vesicles absent due to scratching, usually just non-specific crusts
Term
What auto-antibodies are involved in Dermatitis Herpetiformis?
Definition
Transglutaminase 3
Term
How does Dermatitis Herpetiformis appear histologically?
Definition
SUBepidermal blister with neutrophils AND
eosinophils in upper dermis
Term
How is Dermatitis Herpetiformis treated?
Definition
Gluten free diet and Dapsone
Term
What are the characteristics of delayed type hypersensitivity reactions?
Definition
Does not occur in all individuals, first exposure does not elicit reaction, requires period of sensitization, shape of lesions can be geometric or bizarre
Term
What are some common causes of allergic contact dermatitis?
Definition
Nickel, neomycin, balsam of peru, fragrance mix, etc., location of lesion is localized to area of contact
Term
What is the number one allergen that causes allergic contact dermatitis?
Definition
Nickel sulfate, then neomycin, balsam of peru, and fragrance mixes (advise patients to look for fragrance free products, not just unscented)
Term
What is Rhus Dermatitis?
Definition
Allergic contact dermatitis caused by poison ivy, poison oak, or poison sumac
Term
What is phytophotodermatitis?
Definition
Phototoxic reaction caused by plant that comes into contact with the skin and is then exposed to light (UVA), furocoumarins is the photosensitizer in many plants
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