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Immunobullous Dis.

Immunobullous Diseases

Skin Immune System (SIS) components ? * Langerhans cells -- Keratinocytes -- T and B lymphocytes -- Macrophages -- Mast cells and other cell types
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
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)
**Immunobullous Diseases** **ALL have Blisters from Antibodies that Immunofluorescence
Intraepidermal Immunobullous Diseases = ? * Pemphigus (most serious of the diseases)
Subepidermal Immunobullous Diseases = ? * Bullous Pemphigoid (from IgG and complement) .....* Epidermolysis bullosa acquisita – (assoc. with IBD).... * Dermatitis herpetiformis (IgA and Celiac Disease)
Pemphigus basics = ? * See high in the Mediterranean and Jewish pop..... * Is the only epidermal one.... * See blisters on the skin and mucous membranes
Pemphigus MoA = ? * Autoantibodies against keratinocyte antigens, components of the desmosomes
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
What Immunofluorescence will show ? * Greenish IgG fluorescence with a "Chicken Wire" appearance
Bullous Pemphigoid = ? * subepidermal IgG and complement cause this ... * Don't pop as easily like Pemhigus.... * NO Oral involvement ... * See Large Tense blisters
Bullous Pemphigoid MoA = ? * Antibody against keratinocyte antigens (specifically BPAG2 and BPAG1)
Bullous pemphigoid Immunofluorescence will show ? * IgG immunoreactants to the ROOF(epidermal side) of the blister cavity
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
Immunofluorescence of Dermatitis Herpetiformis = ? * IgA deposits at dermal-epidermal junction and see a good number of neutrophils
Epidermolysis bullosa acquisita (EBA) = ? * subepidermal blistering disease usually presenting in 4th to 6th decade.... * MoA = Target antigen is type VII collagen
Immunofluorescence of EBA = ? * See fluorescence of the FLOOR (roof = bullous pemp.) b/c of the main target, which is Type VII collagen
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)
One that is most fatal if left untreated = ? * Pemphigus, bc of sepsis
Created by: thamrick800