Pemphigus Vulgaris: Update on Etiopathogenesis, Oral Manifestations, and Management

Abstract

ABSTRACT: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.

Desmoglein 3 is overexpressed in inverted papilloma and squamous cell carcinoma of sinonasal cavity

“…10 A previous study using cDNA expression analysis revealed that DSG3 was selectively expressed in lung SCC, whereas normal lung and adenocarcinoma showed low expression levels. 7 In consideration of similar morphological features and disease patterns between the lung and nasal cavity, we focused on DSG3 to identify its role in malignant transformation of sinonasal IP.…”

Gingival lesions as a first symptom of pemphigus vulgaris in pregnancy

“…By exfoliative cytology it is possible to see the Tzanck cells -due to the acantholysis. Direct immunofluorescence is the gold standard for diagnosis 5,11 which shows a reticular pattern at the level of the stratum spinosum due to the deposit of immunoglobins (IgG) and complement C3. Indirect immunofluorescence is 80-90% positive, pointing to the presence of antibodies circulating in the serum.…”

Autoimmune Disorders – Immunopathogenesis and Potential Therapies

“…5,6 A combination of corticosteroids, immunosuppressive agents, pulse therapy, photophoresis and plasmaphoresis may reduce its progression. 7 Early lesions in the mouth can be best attended by appropriate periodontal therapy with maintenance of optimal oral hygiene. High grade lesions are treated by systemic glucocorticoids.…”