Serum levels of autoantibodies to desmoglein 3 in patients with therapy-resistant pemphigus vulgaris successfully treated with adjuvant intravenous immunoglobulins

Acta Derm Venereol. 2004;84(1):48-52. doi: 10.1080/00015550310005861.


The mainstay of treatment of pemphigus vulgaris is systemic corticosteroids. Intravenous immunoglobulins have been reported as an adjuvant corticosteroid-sparing regimen in recalcitrant pemphigus vulgaris. The purpose of the study was to monitor disease activity, serum levels of autoantibodies and doses of oral corticosteroids in 4 patients with recalcitrant pemphigus vulgaris adjuvantly treated with intravenous immunoglobulins (2 g kg(-1) monthly). After initiation of intravenous immunoglobulins, all patients showed clinical improvement and a decrease in autoantibody serum levels, as detected by both indirect immunofluorescence microscopy and ELISA. Corticosteroids and immunosuppressants could be reduced and even discontinued in one patient. In 3 patients, intravenous immunoglobulins were discontinued after 12 cycles. Subsequently, new blisters developed and autoantibody levels rose again. After re-initiation of intravenous immunoglobulins, in 2 patients, the condition quickly improved again, along with a decrease in autoantibody serum levels. It is concluded that the administration of intravenous immunoglobulins was associated with a decrease in circulating autoantibodies and clinical improvement in our patients.

MeSH terms

  • Administration, Oral
  • Adrenal Cortex Hormones / administration & dosage
  • Adult
  • Aged
  • Autoantibodies / blood*
  • Cadherins / immunology*
  • Desmoglein 3
  • Drug Resistance
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Middle Aged
  • Pemphigus / immunology*
  • Pemphigus / therapy*


  • Adrenal Cortex Hormones
  • Autoantibodies
  • Cadherins
  • DSG3 protein, human
  • Desmoglein 3
  • Immunoglobulins, Intravenous