Page 1231 - Veterinary Immunology, 10th Edition
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     Skin Basement Membrane Diseases
  VetBooks.ir  A second set of blistering diseases is associated with the
               development of autoantibodies against the skin basement
               membrane. As a result, affected dogs develop subepidermal bullae.
               Several such diseases have been identified in dogs and other
               domestic animals. They include bullous pemphigoid, linear IgA
               dermatosis, and epidermolysis bullosa acquisita.
               Bullous Pemphigoid
               Bullous pemphigoid is a rare skin disease that resembles
               pemphigus vulgaris. Collies, Shetland Sheepdogs, and Dobermans
               appear to be predisposed to it. It has also been described in
               humans, pigs, horses, and cats. Multiple bullae develop around
               mucocutaneous junctions and in the groin and axillae. However,
               the disease differs from pemphigus vulgaris in that the bullae
               develop in the subepidermis (and are therefore less likely to
               rupture). They tend to be filled with fibrin as well as mononuclear
               cells or eosinophils, and they heal spontaneously. Bullous
               pemphigoid results from the development of autoantibodies
               against type XVII collagen. This molecule is a component of
               hemidesmosomes, the structures that attach basal keratinocytes to
               the basement membrane (Fig. 37.8). The presence of IgG on the
               basement membrane may be demonstrated by immunofluorescence
               that reveals intense linear staining. The prognosis of bullous
               pemphigoid is usually poor, but mild cases may recover after
               treatment with corticosteroids. More commonly, aggressive
               treatment such as high doses of prednisolone, supplemented if
               necessary with cyclophosphamide, azathioprine, and chlorambucil,
               may be needed. Some dogs may develop a bullous pemphigoid-like
               disease in response to autoantibodies against the basement
               membrane protein laminin-5.
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