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Chapter 123 : Skin Diseases
Authors:
Carlos H. Nousari,
Grant J. Anhalt
Content Type: Reference
Publication Year:
2006
Category: Immunology
Book DOI:
10.1128/9781555815905
Chapter DOI:
10.1128/9781555815905.ch123
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Skin Diseases, Page 1 of 2
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Abstract:
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.
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
Figures
Skin Diseases
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Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123fig01
FIGURE 1
(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.
10.1128/9781555815905/f1092-01_thmb.gif
10.1128/9781555815905/f1092-01.gif
FIGURE 1
(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.
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123fig02
FIGURE 2
(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.
10.1128/9781555815905/f1093-01_thmb.gif
10.1128/9781555815905/f1093-01.gif
FIGURE 2
(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.
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123fig03
FIGURE 3
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.
10.1128/9781555815905/f1094-01_thmb.gif
10.1128/9781555815905/f1094-01.gif
FIGURE 3
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.
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123fig04
FIGURE 4
(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.
10.1128/9781555815905/f1095-01_thmb.gif
10.1128/9781555815905/f1095-01.gif
FIGURE 4
(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.
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123fig05
FIGURE 5
DIF of DH showing characteristic granular IgA deposits along the BMZ, with accentuation at the tip of the dermal papillae and microfibrils.
10.1128/9781555815905/f1096-01_thmb.gif
10.1128/9781555815905/f1096-01.gif
FIGURE 5
DIF of DH showing characteristic granular IgA deposits along the BMZ, with accentuation at the tip of the dermal papillae and microfibrils.
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123fig06
FIGURE 6
(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).
10.1128/9781555815905/f1097-01_thmb.gif
10.1128/9781555815905/f1097-01.gif
FIGURE 6
(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).
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123fig07
FIGURE 7
(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.
10.1128/9781555815905/f1097-02_thmb.gif
10.1128/9781555815905/f1097-02.gif
FIGURE 7
(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.
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
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Tables
Skin Diseases
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Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123tab01
TABLE 1
Antibody profiles in pemphigus variants
TABLE 1
Antibody profiles in pemphigus variants
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123
/content/10.1128/9781555815905.ch123.ch123tab02
TABLE 2
Plakin proteins
TABLE 2
Plakin proteins
Citation:
Nousari C, Anhalt G.
2006.
Skin Diseases,
p 1091-1100.
In
Detrick B, Hamilton R, Folds J (ed),
Manual of Molecular and Clinical Laboratory Immunology, 7th Edition.
ASM Press, Washington, DC.
doi: 10.1128/9781555815905.ch123