Figure 2
Immunological and clinical characteristics of autoimmune bullous skin disorders.
(A–C) Pemphigus is characterized by the presence of IgG (and occasionally IgA) specific for desmosomal target antigens (visualized as an intercellular staining pattern by direct immunofluorescence; A) resulting in a loss of intraepidermal adhesion (as shown by histopathology; B) and blisters and/or erosions of the mucous membranes and skin (C). (D–F) In the pemphigoids, including linear IgA bullous dermatosis, IgG (or IgA) autoantibodies bind to antigens of hemidesmosomes and the lamina lucida of the dermoepidermal junction (D), resulting in a loss of subepidermal adhesion (E) and tense blisters (F). (G–I) Epidermolysis bullosa acquisita is associated with IgG (and sometimes IgA) binding to the anchoring fibrils underneath the lamina densa of the dermoepidermal junction (G), resulting in a subepidermal loss of adhesion (H) and tense blisters with a tendency toward scarring and milia formation (I). (J–L) Dermatitis herpetiformis is associated with deposits in the papillary dermis (dotted line indicates the dermoepidermal junction) of IgA reactive with epidermal transglutaminase (J), a subepidermal loss of adhesion (K), and herpetiform blisters or pruritic papules (L). Magnification, ×100 (A, B, D, E, G, H, J, and K).
