tailieunhanh - Chapter 055. Immunologically Mediated Skin Diseases

A number of immunologically mediated skin diseases and immunologically mediated systemic disorders with cutaneous manifestations are now recognized as distinct entities with consistent clinical, histologic, and immunopathologic findings. Many of these disorders are due to autoimmune mechanisms. Clinically, they are characterized by morbidity (pain, pruritus, disfigurement) and in some instances by mortality (largely due to loss of epidermal barrier function and/or secondary infection). The major features of the more common immunologically mediated skin diseases are summarized in this chapter (Table 55-1), as are the systemic disorders with cutaneous manifestations. . | Chapter 055. Immunologically Mediated Skin Diseases A number of immunologically mediated skin diseases and immunologically mediated systemic disorders with cutaneous manifestations are now recognized as distinct entities with consistent clinical histologic and immunopathologic findings. Many of these disorders are due to autoimmune mechanisms. Clinically they are characterized by morbidity pain pruritus disfigurement and in some instances by mortality largely due to loss of epidermal barrier function and or secondary infection . The major features of the more common immunologically mediated skin diseases are summarized in this chapter Table 55-1 as are the systemic disorders with cutaneous manifestations. Table 55-1 Immunologically Mediated Blistering Diseases Disease Clinical Histology Immunopatholog y Autoantigens3 Pemphigus foliaceus Crusts and shallow erosions on scalp central face upper chest and back Acantholytic blister formed in superficial layer of epidermis Cell surface deposits of IgG on keratinocytes Dsg1 Pemphigus vulgaris Flaccid blisters denuded skin oromucosal lesions Acantholytic blister formed in suprabasal layer of epidermis Cell surface deposits of IgG on keratinocytes Dsg3 plus Dsg1 in patients with skin involvement Paraneoplasti c pemphigus Painful stomatitis with papulosquamou s or lichenoid eruptions that progress to blisters Acantholysis keratinocyte necrosis and vacuolar interface dermatitis Cell surface deposits of IgG and C3 on keratinocytes and variably similar immunoreactants in epidermal BMZ Plakin protein family members and desmosomal cadherins see text for details Bullous pemphigoid Large tense blisters on flexor surfaces Subepiderm al blister with eosinophil- Linear band of IgG and or C3 in epidermal BMZ BPAG1 BPAG2 and trunk rich infiltrate Pemphigoid gestationis Pruritic urticarial plaques rimmed by vesicles and bullae on the trunk and extremities Teardropshaped subepiderma l blisters in dermal papillae eosinophilrich infiltrate

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