Identical HLA class II alleles predispose to drug-triggered and idiopathic pemphigus vulgaris

Acta Derm Venereol. 1995 Jan;75(1):12-4. doi: 10.2340/00015555751214.


In pemphigus vulgaris, a dermatological autoimmune disease, specific human leukocyte antigen (HLA) class II alleles, DR4 (DRB1*0402) and DRw14 (DRB1*1401, in linkage disequilibrium with DQB1*0503), are thought to be susceptibility genes involved in the onset of the disease. We studied the HLA class II alleles (DQA1, DQB1, DRB1 and DPB1) of 6 patients with pemphigus, in whom the disease was "triggered" by drugs containing sulphydryl or another sulphur-containing group. All patients carried the DRB1*0402 susceptibility allele, and one patient also carried the second susceptibility allele, namely DQB1*0503 (in linkage with DRB1*1401). Bacterial, viral or environmental agents are suspected to trigger the onset of autoimmune diseases. Our study demonstrated the presence in patients with drug-triggered pemphigus vulgaris of the same HLA alleles thought to predispose to idiopathic pemphigus vulgaris. This finding strengthens the notion that these HLA alleles may be true disease susceptibility genes.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Alleles*
  • Autoimmune Diseases / genetics*
  • Disease Susceptibility
  • Female
  • Genetic Linkage / genetics
  • HLA Antigens / genetics*
  • HLA-DP Antigens / genetics
  • HLA-DP beta-Chains
  • HLA-DQ Antigens / genetics
  • HLA-DQ alpha-Chains
  • HLA-DQ beta-Chains
  • HLA-DR Antigens / genetics
  • HLA-DR Serological Subtypes
  • HLA-DR4 Antigen / genetics
  • HLA-DRB1 Chains
  • Humans
  • Male
  • Middle Aged
  • Pemphigus / chemically induced
  • Pemphigus / genetics
  • Pemphigus / immunology*
  • Sulfhydryl Compounds / adverse effects
  • Sulfur / adverse effects


  • HLA Antigens
  • HLA-DP Antigens
  • HLA-DP beta-Chains
  • HLA-DPB1 antigen
  • HLA-DQ Antigens
  • HLA-DQ alpha-Chains
  • HLA-DQ beta-Chains
  • HLA-DQA1 antigen
  • HLA-DQB1 antigen
  • HLA-DR Antigens
  • HLA-DR Serological Subtypes
  • HLA-DR14
  • HLA-DR4 Antigen
  • HLA-DRB1 Chains
  • HLA-DRB1*04:02 antigen
  • Sulfhydryl Compounds
  • Sulfur