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Immunobullous Diseases

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Question
Answer
Skin Immune System (SIS) components ?   * Langerhans cells -- Keratinocytes -- T and B lymphocytes -- Macrophages -- Mast cells and other cell types  
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What are the role of the Langerhans Cells in the SIS ?   * Dendritic shaped cells that help in Antigen Presentation to T-Cells to activate the adaptive immune response...... * They are good at presenting bc they have MHC Class I & II, CD40, CAMs, and B7  
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What is the role of keratinocytes in the immunologic/inflammatory skin response ?   * Become activated, and respond by producing PG's and Pro-Inflam cyotkines (TNF, IL-1,6,8)  
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**Immunobullous Diseases**   **ALL have Blisters from Antibodies that Immunofluorescence  
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Intraepidermal Immunobullous Diseases = ?   * Pemphigus (most serious of the diseases)  
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Subepidermal Immunobullous Diseases = ?   * Bullous Pemphigoid (from IgG and complement) .....* Epidermolysis bullosa acquisita – (assoc. with IBD).... * Dermatitis herpetiformis (IgA and Celiac Disease)  
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Pemphigus basics = ?   * See high in the Mediterranean and Jewish pop..... * Is the only epidermal one.... * See blisters on the skin and mucous membranes  
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Pemphigus MoA = ?   * Autoantibodies against keratinocyte antigens, components of the desmosomes  
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The 2 Types of Pemphigus = ?   * Pemphigus vulgaris = More severe and see on Skin AND Mucous membranes..... * Pemphigus foliaceus = skin issues and are a little more superficial blisters that involve autoantibodies are against Dsg1, NOT 1 and 3 like Vulgaris  
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What Immunofluorescence will show ?   * Greenish IgG fluorescence with a "Chicken Wire" appearance  
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Bullous Pemphigoid = ?   * subepidermal IgG and complement cause this ... * Don't pop as easily like Pemhigus.... * NO Oral involvement ... * See Large Tense blisters  
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Bullous Pemphigoid MoA = ?   * Antibody against keratinocyte antigens (specifically BPAG2 and BPAG1)  
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Bullous pemphigoid Immunofluorescence will show ?   * IgG immunoreactants to the ROOF(epidermal side) of the blister cavity  
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Dermatitis Herpetiformis basics = ?   * Intensely pruritic vesicles and see NO mucousal involvement.... * Lesions may appear as grouped symmetrically vesicles..... * Associated with CELIAC Disease, so if you see these, check for gluten insensitivity  
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Immunofluorescence of Dermatitis Herpetiformis = ?   * IgA deposits at dermal-epidermal junction and see a good number of neutrophils  
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Epidermolysis bullosa acquisita (EBA) = ?   * subepidermal blistering disease usually presenting in 4th to 6th decade.... * MoA = Target antigen is type VII collagen  
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Immunofluorescence of EBA = ?   * See fluorescence of the FLOOR (roof = bullous pemp.) b/c of the main target, which is Type VII collagen  
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Some Other Bullous Diseases = ?   * Mucous membrane pemphigoid -- Pemphigoid gestationis (pregnant ppl) -- Linear IgA bullous disease (similar to Derm. Herpat., but is NOT associated with Celiac Disease)  
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One that is most fatal if left untreated = ?   * Pemphigus, bc of sepsis  
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