Term
| What is vesiculobullous disease? |
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Definition
| Disease state characterized by persistent formation of either vesicles or bullae |
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Term
| What are the major autoimmune blistering diseases? |
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Definition
| Pemphigus, bullous pemphigoid, linear IgA bullous dermatoses, Dermatitis Herpetiformis |
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Term
| What causes autoimmune blistering diseases? |
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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" |
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Term
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Definition
| When skin cells become "unglued." Location depends on the adhesion proteins involved. Desmosomes = epidermis, Hemidesmosomes = subepidermal. Location correlates with type of disease |
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Term
| Pemphigus is what type of autoimmune blistering disease? |
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Definition
| Intraepidermal, PV is above basal layer of epidermis, PF is below corneal layer of epidermis |
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Term
| Bullous Pemphigoid is what type of autoimmune blistering disease? |
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Definition
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Term
| What is a flaccid blister? |
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Definition
| Fragile blister that is easily ruptured, indicates intraepidermal blister that is higher up |
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Term
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Definition
| Firm blister not easily ruptured, indicates deeper subepidermal blister |
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Term
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Definition
| Pressing lightly on the edge of the blister causes superficial skin to tear away, indicates fragile blister, seen in Phemphigus but not BP |
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Term
| What is a Asboe-Hanson sign? |
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Definition
| Pressing lightly on the blister causes fluid to spread into surrounding intact skin, seen in Phemphigus but not BP |
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Term
| How are blisters biopsied? |
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Definition
| Punch biopsy and then examination under light microscope with H&E staining to determine which layer is affected, then sent for direct immunofluourescence |
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Term
| What are the different presentations of disease under direct immunofluorescence? |
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Definition
| Dermatitis Herpetiformis = granular deposition of IgA, Linear IgA = linear deposition of IgA, bullous pemphigoid = linear deposition of IgG |
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Term
| What are the characteristics of Pemphigus Vulgaris |
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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 |
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Term
| How does Pemphigus Vulgaris present? |
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Definition
| All patients have oral mucosal lesions which precede skin lesions, characteristically flaccid blisters in upper epidermis, heals with brown hyperpigmentation w/out scarring |
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Term
| What auto-antibodies are involved in Pemphigus Vulgaris? |
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Definition
| Desmoglein 3 only in mucosal dominant, Desmoglein 1 and 3 in mucocutaneous |
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Term
| How does Pemphigus Vulgaris present histologically? |
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Definition
Intraepidermal blister w/ suprabasilar acantholysis and blister with
“tombstoning” of the basal layer |
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Term
| How is Pemphigus Vulgaris treated? |
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Definition
| Aimed at stopping the production of autoantibodies via corticosteroids and immunosuppressants |
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Term
| What is the clinical presentation of Pemphigus Foliaceous? |
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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 |
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Term
| What auto-antibodies are involved in Pemphigus Foliaceous? |
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Definition
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Term
| How do you differentiate Pemphigus Foliaceous from Pemphigus Vulgaris? |
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Definition
| In Pemphigus Foliaceous, blister occurs higher up epidermis, desmoglobin 1 only, does not involve mucous membrane |
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Term
| What is the most common type of autoimmune blistering disease? |
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Definition
| Bullous Pemphoid, causes subepidermal blistering and most commonly occurs in the elderly |
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Term
| How does Bullous Pemphoid present clinically? |
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Definition
| Widespread TENSE blisters w/good structural integrity, can be very pruritic, negative Nikolsky sign |
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Term
| What medications are recommended for treating Bullous Pemphigoid? |
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Definition
| Lasix (furosemide) and Sulfa based drugs such as captopril, penicillamine, and gold |
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Term
| What auto-antibodies are involved in Bullous Pemphigoid? |
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Definition
| Directed against components of hemidesmosomes, specifically BP Ag1 (BP230) and BP Ag2 (BP180) |
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Term
| How is Bullous Pemphigoid treated? |
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Definition
| orticosteroids and immunosuppressants aimed at decreasing antibody response |
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Term
| How does Bullous Pemphigoid present histologically? |
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Definition
| Subepidermal blister w/numerous eosinophils, Ab's (IgG or complement C3) fluoresce in a linear "roof" pattern upon direct immunofluourescence |
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Term
| What are the characteristics of Linear IgA Bullous Dermatosis? |
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Definition
| Autoimmune subepidermal blistering disorder w/vesicles and bullae (some are annular), lesions are variable presentation |
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Term
| What causes Linear IgA Bullous Dermatosis? |
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Definition
| Often drug induced in adults |
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Term
| How is Linear IgA Bullous Dermatosis treated? |
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Definition
| Dapsone, Sulfapyridine, Oral Corticosteroids |
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Term
| What are the characteristics of Dermatitis Herpetiformis? |
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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 |
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Term
| What auto-antibodies are involved in Dermatitis Herpetiformis? |
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Definition
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Term
| How does Dermatitis Herpetiformis appear histologically? |
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Definition
SUBepidermal blister with neutrophils AND
eosinophils in upper dermis |
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Term
| How is Dermatitis Herpetiformis treated? |
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Definition
| Gluten free diet and Dapsone |
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Term
| What are the characteristics of delayed type hypersensitivity reactions? |
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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 |
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Term
| What are some common causes of allergic contact dermatitis? |
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Definition
| Nickel, neomycin, balsam of peru, fragrance mix, etc., location of lesion is localized to area of contact |
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Term
| What is the number one allergen that causes allergic contact dermatitis? |
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Definition
| Nickel sulfate, then neomycin, balsam of peru, and fragrance mixes (advise patients to look for fragrance free products, not just unscented) |
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Term
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Definition
| Allergic contact dermatitis caused by poison ivy, poison oak, or poison sumac |
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Term
| What is phytophotodermatitis? |
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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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