Term
| MMP is associated with the presence of circulating autoantibodies that target various antigens of the basement membrane zone (BMZ). What are these autoantibodies? |
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Definition
Epidermal (salt-split): Collagen XVII (BP180), BPAG1e (BP230), Integrin α6/β4
Dermal (salt-split): Laminin-332, Collagen VII (rare involvement)
These are NOT specific enough for a diagnosis of MMP (other AISBDs may have them) and not all affected patients will have detectable circulating autoantibodies... |
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Term
| What are the typical clinical signs of MMP? What body sites are most often involved? |
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Definition
Symmetrical skin lesions including erosions and ulcers, crusting, erythema, vesicles/bullae, scarring, & hypopigmentation
Affetced body regions include oral/perioral (most common initial site of lesion development), ocular/periocular, nasal, genital & concave pinnae
100% will exhibit lesions on 1 or more (2+ is common) mucosae or mucocutaneous junctions (the nasal
planum = a modified mucosa)
Haired skin involvement = less common |
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Term
| What breed appears to be predisposed to the development of canine MMP? |
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Definition
| Middle aged German Sheps (big surprise!) |
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Term
| Several review articles have assessed that complete resolution of the clinical signs of MMP is most likely to be achieved with what drug/combination of drugs? |
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Definition
2016 review = CR in 60% of dogs that
received tetracycline antibiotics and niacinamide alone or
in combination with another immunosuppressive drug
2004 review = 46% if dogs had a partial or complete resolution of clinical signs with a combination of tetracycline
and niacinamide
These drugs should be considered as a first-line treatment
for canine MMP...GCs (must be part of a combo) and other steroid sparing agents may also be appropriate |
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Term
| Which autoantibodies characterize canine linear IgA and what is their target? |
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Definition
| IgA/IgG autoantibodies that recognize epitopes on the processed extracellular segment of type XVII collagen (LAD-1) |
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Term
| What is the molecular target of EBA? |
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Definition
| The NC terminal domain of collagen VII (Great Danes) |
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Term
| Canine BP: Skin dominant AISBD- what are the major and minor target antigens? |
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Definition
-Major target antigen: NC16A domain of col XVII
-Minor target antigen: BP230
-Dermo-epidermal separation with inflammatory infiltrate (eosinophils seen in 67% and neutrophils in 71% of dogs)
-Circulating anti-BMZ IgG and IgE detected in 100% and 60% |
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Term
BP and anti-collagen XVII autoimmunity is most common in what breed of pigs?
What type of lesions develop in horses with BP? |
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Definition
-Mild disease in young Yucatan mini-pigs
-Severe blistering (mucosa + skin) in 3 horses |
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Term
| What are the functions of Laminin 332? |
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Definition
-Laminin 332 is part of anchoring filaments as well as focal adhesions
-Adhesion
-Cell growth and proliferation (growth factors binding sites within LG domains
-recruitment of other BMZ members during wound healing
-antimicrobial peptide (cleaved LG4) |
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Term
| Laminin 332 causes what congenital and autoimmune skin pathology? |
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Definition
Congenital: junctional EB (dog, cat, horse, cattle, sheep)
Autoimmune: MMP (BP180>>> lam332), junctional EBA, mixed AISBD |
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Term
| What are the keratin expression patterns? What keratins cause proliferation activation and are present in the pawpad? |
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Definition
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Term
| Collagen XVII (BP180) targets and what autoimmune disease results? |
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Definition
-Congenital: none recognized in vet medicine
-Autoimmune: MMP, BP, LAD
[image] |
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Term
| What AISBDs have anti-laminin 332 autoantibodies? |
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Definition
-MMP (less common than BP180)
-Mixed AISBD: 3 dogs reported, 2.5-4 year age of onset with prominent haired skin involvement with oral cavity lesions and concurrent anti-collagen VII IgG
-Junctional EBA: 5 dogs, 6m-8 year age of onset with haired skin, footpads, and oral mucosa (100%) with clinically resembling EBA |
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Term
| Localization of split in relationship to Laminin |
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Definition
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Term
| Name the auto-antibody target identified in canine epidermolysis bullosa acquisita. |
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Definition
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