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Grand Rounds |

Pemphigus—Diseases of Antidesmosomal Autoimmunity

Mark C. Udey, MD, PhD; John R. Stanley, MD
JAMA. 1999;282(6):572-576. doi:10.1001/jama.282.6.572.
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Figure 1. Clinical and Histologic Features of Pemphigus
Graphic Jump Location
A, Stomatitis in a patient with pemphigus vulgaris (PV). B, Erosions typical of PV. C, Histology of a PV skin lesion revealing suprabasilar acantholysis (hematoxylin-eosin stain, original magnification ×40). D, Indirect immunofluorescence testing of PV serum sample showing diagnostic antikeratinocyte IgG reactivity (monkey esophagus as substrate). E, Crusted erythematous skin lesions of pemphigus foliaceus. F, Histology of a pemphigus foliaceus lesion demonstrating the characteristic subcorneal intraepidermal cleavage (hematoxylin-eosin, original magnification × 40).
Figure 2. Structural Features of Desmosomes
Graphic Jump Location
Schematic representation of the current consensus view of protein-protein interactions in desmosomes. Many desmosomal components (including other members of the plakin family) are not depicted because their physical relationships to other desmosomal components are not yet well characterized.15Dsg indicates desmogleins.
Figure 3. The Desmoglein Compensation Hypothesis
Graphic Jump Location
Pemphigus foliaceus sera contain only anti–desmoglein (Dsg) 1 antibodies and cause blisters by interfering with the function of Dsg1, where there is no Dsg3 to compensate, namely, in the superficial epidermis of skin. In early pemphigus vulgaris, when patients produce only anti-Dsg3 antibodies, separations develop deep in mucous membranes, where Dsg1 will not compensate for loss of Dsg3-mediated adhesion. Later in the course of pemphigus vulgaris, when the patients' sera contain both anti-Dsg1 and anti-Dsg3 antibodies, the function of both Dsgs is compromised and blisters occur in skin and mucous membranes. Arrow indicates that the antibody is capable of causing blisters; X, blister formation is blocked by the compensating Dsg. The triangles represent the distribution of Dsg1 and Dsg3.



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