The immunopathology of bullous pemphigoid |
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| Authors: | M J Imber G F Murphy R E Jordon |
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| Affiliation: | 1. From the Departments of Pathology, Massachusetts General Hospital and Brigham and Women''s Hospital, Harvard Medical School, Boston, Massachusetts USA;2. The Department of Dermatology, The University of Texas, Health Science Center, Houston, Texas USA;1. Servicio de Anatomía Patológica, Hospital Álvaro Cunqueiro, Vigo, Spain;2. Clínica Pérez & Gavín, Vigo, Spain;3. Servicio de Dermatología, Hospital do Meixoeiro, Vigo, Spain;1. Department of Dermatology, Rouen University Medical Center, France;2. Department of Anatomical Pathology and Cytology, Rouen University Medical Center, France;1. Department of Dermatology, Henry Ford Health System, Detroit, Michigan;2. Department of Dermatology & Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania;1. Departamento de Dermatología, Universidad de Pittsburgh, Pittsburgh, Pensilvania, EE. UU.;2. Horizon Dermatology, UPMC, Hermitage, Pensilvania, EE. UU.;1. Department of Dermatology, INSERM U1234, Rouen University Hospital, University of Rouen, France;2. Department of Immunology, Rouen University Hospital, University of Rouen, France;3. Department of Gynecology and Obstetrics, Rouen University Hospital, University of Rouen, France |
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| Abstract: | The immunopathology of bullous pemphigoid features basement membrane zone (BMZ) deposition of immunoglobulin and complement and the presence of circulating immunoreactants.1,2 The demonstration of linear subepidermal deposition of C3 and/or IgG is usually necessary to establish the diagnosis. By light microscopy, fully evolved lesions show subepidermal BMZ separation and variable infiltrates of eosinophils, mononuclear cells, and neutrophils. Other bullous disorders that share overlapping histopathologic and immunopathologic findings include cicatricial pemphigoid, herpes gestationis, linear IgA bullous dermatosis, dermatitis herpetiformis, and epidermolysis bullosa acquisita.3,4 The application of immunofluorescence techniques has demonstrated the immune nature of many of these disorders. Accordingly, bullous pemphigoid was rendered unique from the pemphigus group blistering diseases that features intra-epidermal deposition of immunoreactants. More recently, mono-clonal antibody probes have allowed detailed study of the role of immunoglobulin subtypes and complement complexes and cofactors in the immunopathology of bullous pemphigoid. |
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