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Immunobullous Dis.
Immunobullous Diseases
| Question | Answer |
|---|---|
| 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
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