Chapter 055. Immunologically Mediated Skin Diseases (Part 1)

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Harrison's Internal Medicine Chapter 55. Immunologically Mediated Skin Diseases Immunologically Mediated Skin Diseases: Introduction 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). ...

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Chapter 055. Immunologically Mediated Skin Diseases (Part 1) Harrison's Internal Medicine > Chapter 55. Immunologically Mediated Skin Diseases Immunologically Mediated Skin Diseases: Introduction 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 Clini Histolo Immunopat Autoanti cal gy hology gensa Pemphig Crust Acantho Cell surface Dsg1 us foliaceus s and lytic blister deposits of IgG on shallow formed in keratinocytes erosions on superficial scalp, layer of central face, epidermis upper chest, and back Pemphig Flacci Acantho Cell surface Dsg3 us vulgaris d blisters, lytic blister deposits of IgG on (plus Dsg1 in denuded formed in keratinocytes patients with skin, suprabasal skin oromucosal layer of
lesions epidermis involvement) Paraneo Painf Acantho Cell surface Plakin plastic ul stomatitis lysis, deposits of IgG and protein family pemphigus with keratinocyte C3 on keratinocytes members and papulosqua necrosis and and (variably) desmosomal mous or vacuolar similar cadherins (see lichenoid interface immunoreactants in text for details) eruptions dermatitis epidermal BMZ that progress to blisters Bullous Large Subepid Linear band BPAG1, pemphigoid tense blisters ermal blister of IgG and/or C3 in BPAG2 on flexor with epidermal BMZ surfaces and eosinophil-rich trunk infiltrate Pemphig Prurit Teardro Linear band BPAG2 oid gestationis ic, urticarial p-shaped, of C3 in epidermal (plus BPAG1 in plaques, subepidermal BMZ some patients)
rimmed by blisters in vesicles and dermal bullae on the papillae; trunk and eosinophil-rich extremities infiltrate Linear Prurit Subepid Linear band BPAG2 IgA disease ic small ermal blister of IgA in epidermal (see text for papules on with BMZ specific details) extensor neutrophil-rich surfaces; infiltrate occasionally larger, arciform blisters Cicatrici Erosi Subepid Linear band BPAG2, al pemphigoid ve and/or ermal blister of IgG, IgA, and/or laminin 5, or blistering that may or C3 in epidermal others lesions of may not BMZ mucous include a
membranes leukocytic and possibly infiltrate the skin; scarring of some sites Epiderm Bliste Subepid Linear band Type VII olysis bullosa rs, erosions, ermal blister of IgG and/or C3 in collagen acquisita scars, and that may or epidermal BMZ milia on may not sites include a exposed to leukocytic trauma; infiltrate widespread, inflammator y, tense blisters may be seen initially Dermatit Extre Subepid Granular Epiderma
is herpetiformis mely pruritic ermal blister deposits of IgA in l small with dermal papillae transglutaminase papules and neutrophils in vesicles on dermal papillae elbows, knees, buttocks, and posterior neck a Autoantigens bound by these patients' autoantibodies are defined as follows: Dsg1, desmoglein 1; Dsg3, desmoglein 3; BPAG1, bullous pemphigoid antigen 1; BPAG2, bullous pemphigoid antigen 2. Note: BMZ, basement membrane zone.