Pemphigus vulgaris: update on etiopathogenesis, oral manifestations, and management.
Pemphigus is a group of potentially life-threatening diseases characterized by cutaneous and mucosal blistering. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles. Certain ethnic groups, such as Ashkenazi Jews and those of Mediterranean origin, are especially liable to pemphigus. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibodies. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examinations, is essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected by both normal human skin and monkey esophagus or by enzyme-linked immunosorbent assay (ELISA). Before the introduction of corticosteroids, pemphigus vulgaris was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using systemic corticosteroids, with azathioprine, dapsone, methotrexate, cyclophosphamide, and gold as adjuvants or alternatives, but mycophenolate mofetil and intravenous immunoglobulins also appear promising.
Scully
Crispian
C
Department of Oral Medicine, Eastman Dental Institute for Oral Health Care Sciences, University College London, University of London, 256 Gray's Inn Road, London WC1X 8LD, UK. scully.c@eastman.ucl.ac.uk
Challacombe
Stephen J
SJ
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000818:Animals; D001323:Autoantibodies; D001324:Autoantigens; D015820:Cadherins / Q000276:immunology; D051183:Desmoglein 1; D051184:Desmoglein 3; D005006:Ethnicity; D005802:Genes, MHC Class II; D006801:Humans; D007111:Immunity, Cellular; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D019226:Oral Ulcer / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D010392:Pemphigus / Q000150:complications / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology*
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D001324:Autoantigens; D015820:Cadherins; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3; D007166:Immunosuppressive Agents