U1RNP antibody-positive neonatal lupus. A report of two cases with immunogenetic studies

Arch Dermatol. 1992 Nov;128(11):1490-4.

Abstract

Background: Neonatal lupus erythematosus (NLE) is a distinct subset of lupus characterized by cutaneous findings (50%), cardiac conduction defects (50%), and autoantibodies to Ro (SS-A) antigen. HLA typing studies of Ro (SS-A) antibody-positive mothers of infants with NLE have shown an association with the HLA-DR3 phenotype. We report the clinical and serologic features of two infant-mother pairs who are U1RNP antibody positive and Ro (SS-A) antibody negative. HLA typing is reported on these infants, their mothers, and two additional infant-mother pairs with U1RNP antibody-positive lupus whose clinical features have been reported previously.

Observations: Cutaneous findings included malar erythema, annular and polycyclic plaques, and scales that resolved with residual telangiectasia and hyperpigmentation 6 months after birth. Systemic abnormalities, including complete heart block, were absent. HLA typing revealed HLA-DR3 in two of four mothers, HLA-DR4 and HLA-DRw53 in two of four mothers, and either HLA-DQ1 or HLA-DQ3 in four of four mothers. No distinct HLA associations were seen in the three infants examined.

Conclusions: The spectrum of cutaneous disease in U1RNP antibody-positive infants is similar to Ro (SS-A) antibody-positive infants with NLE. Complete heart block was not a feature of U1RNP antibody-positive NLE. HLA typing studies show a more diverse immunogenetic pattern in U1RNP antibody-positive mothers of infants with NLE compared with Ro (SS-A) antibody-positive mothers.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Antinuclear / analysis*
  • Antibodies, Antinuclear / genetics
  • Autoantibodies / analysis*
  • Autoantibodies / genetics
  • Autoantigens / analysis
  • Autoantigens / genetics
  • Female
  • HLA Antigens / analysis*
  • HLA Antigens / genetics
  • HLA-A1 Antigen / analysis
  • HLA-B8 Antigen / analysis
  • HLA-DQ Antigens / analysis
  • HLA-DR Antigens / analysis
  • HLA-DR2 Antigen / analysis
  • HLA-DR3 Antigen / analysis
  • HLA-DR4 Antigen / analysis
  • HLA-DRB4 Chains
  • Humans
  • Immunogenetics
  • Infant
  • Lupus Erythematosus, Systemic / genetics
  • Lupus Erythematosus, Systemic / immunology*
  • Male
  • RNA, Small Cytoplasmic*
  • Ribonucleoprotein, U1 Small Nuclear / genetics
  • Ribonucleoprotein, U1 Small Nuclear / immunology*
  • Ribonucleoproteins / analysis
  • Ribonucleoproteins / genetics
  • Ribonucleoproteins, Small Nuclear*
  • SS-B Antigen
  • Transcription Factors / analysis
  • Transcription Factors / genetics
  • snRNP Core Proteins

Substances

  • Antibodies, Antinuclear
  • Autoantibodies
  • Autoantigens
  • HLA Antigens
  • HLA-A1 Antigen
  • HLA-B8 Antigen
  • HLA-DQ Antigens
  • HLA-DQ1 antigen
  • HLA-DQ3 antigen
  • HLA-DR Antigens
  • HLA-DR2 Antigen
  • HLA-DR3 Antigen
  • HLA-DR4 Antigen
  • HLA-DR53
  • HLA-DRB4 Chains
  • RNA, Small Cytoplasmic
  • RO60 protein, human
  • Ribonucleoprotein, U1 Small Nuclear
  • Ribonucleoproteins
  • Ribonucleoproteins, Small Nuclear
  • SS-A antigen
  • Transcription Factors
  • snRNP Core Proteins