
Full text loading...
Category: Immunology
Skin Diseases, Page 1 of 2
< Previous page | Next page > /docserver/preview/fulltext/10.1128/9781555815905/9781555813642_Chap123-1.gif /docserver/preview/fulltext/10.1128/9781555815905/9781555813642_Chap123-2.gifAbstract:
The dermis and epidermis have a dense network of adhesion proteins and fibrous structural proteins that provide mechanical strength to the skin. A number of these adhesion molecules are known to become targets of autoantibodies, and antigen-antibody interactions in the skin lead to the clinical manifestations of autoimmune bullous skin diseases. Demonstration of the characteristic autoantibodies in the tissue and circulating in the blood is the critical diagnostic feature of numerous such diseases. In most of these diseases, the circulating autoantibodies have also been shown, by passive transfer in animal models, to directly cause the characteristic tissue injury. For each of these diseases, this chapter describes the expected findings in tissue and serum and outlines immunochemical tests that may be useful in diagnosis. There are four major forms of pemphigus: pemphigus vulgaris (PV), pemphigus foliaceus (PF), paraneoplastic pemphigus (PNP), and IgA pemphigus. All forms are characterized by a loss of normal epidermal cell-to-cell adhesion (acantholysis) and by the presence of pathogenic autoantibodies reacting against desmosomal adhesion molecules. Pemphigus is a potentially lethal disease; to establish the diagnosis with certainty, the presence of both tissue-bound and circulating autoantibodies must be demonstrated.
Full text loading...
(Left) DIF of oral mucosa in PV showing linear IgG deposits on the epithelial cell surfaces; (right) DIF of oral mucosa in PNP showing linear IgG deposits on the epithelial cell surfaces as well as along the BMZ.
(Left) IIF in PV showing the presence of circulating IgG autoantibodies binding the epithelial cell surfaces of monkey esophagus substrate with sparing of BMZ and the basal third of the epithelial stratum; (right) IIF in PNP showing the presence of circulating IgG autoantibodies binding the epithelial cell surfaces as well as the BMZ of monkey esophagus substrate.
IIF in PNP showing the presence of circulating IgG autoantibodies binding the epithelial cell surfaces as well as the BMZ of murine bladder, a plakin-rich substrate.
(Left) IIF in BP showing the presence of circulating IgG autoantibodies binding the epidermal side (roof) of human salt-split skin; (right) IIF in EBA showing the presence of circulating IgG autoantibodies binding the dermal side (floor) of human salt-split skin.
DIF of DH showing characteristic granular IgA deposits along the BMZ, with accentuation at the tip of the dermal papillae and microfibrils.
(Left) DIF of skin of IgA vasculitis (Henoch-Schoenlein purpura) showing granular IgA deposits in and around superficial papillary dermal vessels; (right) DIF of skin in hypocomple-mentemic urticarial vasculitis showing granular IgG deposits in and around superficial papillary dermal vessels as well as along the BMZ (lupus band-like).
(Left) DIF of skin in discoid LE showing dense granular IgM deposits along the follic-ular (adnexal) BMZ; (right) DIF of skin in systemic LE showing granular IgG deposits along the epidermal BMZ.
Antibody profiles in pemphigus variants
Plakin proteins