Number II. Pemphigus vulgaris.
Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering. Pemphigus vulgaris (PV), the most common variant, is characterized by circulating IgG antibodies directed against desmoglein 3 (Dsg3), with about half the patients also having Dsg1 autoantibodies. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles and ethnic groups such as Ashkenazi Jews and those of Mediterranean and Indian origin, are especially liable. 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 examination are essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected using both normal human skin and monkey oesophagus or by enzyme-linked immunosorbent assay. Before the introduction of corticosteroids, PV was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using corticosteroids, with azathioprine or other adjuvants or alternatives but newer therapies with potentially fewer adverse effects, also appear promising.
Black
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Guys and St Thomas' Hospital Medical School, Kings College, London, UK.
Mignogna
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MD
Scully
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C
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use; D001768:Blister / Q000628:therapy; D015820:Cadherins / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000276:immunology; D051183:Desmoglein 1; D051184:Desmoglein 3; D051182:Desmogleins; D051180:Desmoplakins; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009059:Mouth Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012074:Remission Induction
D000305:Adrenal Cortex Hormones; D015820:Cadherins; D003598:Cytoskeletal Proteins; C495297:DSG1 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3; D051182:Desmogleins; D051180:Desmoplakins; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D001379:Azathioprine