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Pemphigoid: Clinical, Histologic, Immunopathologic, and Therapeutic Considerations

Kim B. Yancey, MD; Conleth A. Egan, MB, MRCPI
JAMA. 2000;284(3):350-356. doi:10.1001/jama.284.3.350.
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Autoimmune blistering diseases are generally distinct entities characterized by relatively consistent clinical, histologic, and immunopathologic findings. These disorders may cause impaired adhesion of epidermis to epidermal basement membrane (eg, the pemphigoid group of disorders [bullous, gestational, and mucous membrane]) or impaired adhesion of epidermal cells to each other (eg, the pemphigus group of disorders). Recent studies have shown that these disorders are characterized by autoantibodies that often display pathogenic (ie, blister-forming) activity in passive transfer models. Interestingly, the autoantigens targeted by these patients' autoantibodies represent important structural proteins that promote cell matrix (eg, pemphigoid) or cell-to-cell (eg, pemphigus) adhesion in skin. Autoimmune blistering diseases are characterized by substantial morbidity (pruritus, pain, disfigurement), and in some instances, mortality (secondary to loss of epidermal barrier function). Treatment with systemic immunosuppressives has reduced morbidity and mortality in patients with these diseases.

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Figures

Figure 1. Clinical, Histologic, and Immunopathologic Features of Bullous, Gestational, and Mucous Membrane Pemphigoid
Graphic Jump Location
A, Tense blisters situated on inflamed and noninflamed skin along with crusted erosions in a patient with bullous pemphigoid. B, Urticarial plaques rimmed by vesicles, blisters, and erosions in a patient with pemphigoid gestationis. (Reproduced with permission from Yancey KB, Lawley TJ. Herpes gestationis. In: Theirs BH, Dobson RL, eds. Pathogenesis of Skin Disease. New York, NY: Churchill Livingstone; 1986:185.) C, Conjunctival injection and cicatrization in a patient with mucous membrane pemphigoid. D, Light microscopy of lesional skin from a patient with bullous pemphigoid demonstrates a subepidermal blister with fibrin and a leukocytic infiltrate (hematoxylin-eosin, original magnification × 40). E, Direct immunofluorescence microscopy of perilesional skin from a patient with bullous pemphigoid demonstrates in situ continuous linear deposits of IgG in the epidermal basement membrane (original magnification × 50). F, Indirect immunofluorescence microscopy demonstrates the reactivity of circulating IgG from a patient with bullous pemphigoid against the epidermal side of a 1 molar salt-split skin test substrate (original magnification × 50).
Figure 2. Schematic Overview of the Epidermal Basement Membrane
Graphic Jump Location
A, Electron microscopy studies of skin demonstrate that basal keratinocytes overlie the lamina lucida, which is in turn positioned just above the lamina densa (ie, the basement membrane proper) and superficial dermis. B, Ultrastructurally, hemidesmosome-anchoring filament-attachment complexes bind the cytoskeleton of basal keratinocytes to the underlying lamina densa, anchoring fibrils, and fibrillar elements (eg, interstitial collagen and elastin fibers) within the superficial dermis. The dominant regions within epidermal basement membrane that are targeted by IgG autoantibodies in patients with pemphigoid diseases and epidermolysis bullosa acquisita are indicated by brackets.

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