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A Report of Two Cases With Immunogenetic Studies

Elizabeth M. Dugan, MD; Walter W. Tunnessen, MD; Paul J. Honig, MD; Rosemarie M. Watson, MB, MRCPI

Arch Dermatol. 1992;128(11):1490-1494.

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.

(Arch Dermatol. 1992;128:1490-1494)

Author Affiliations
From the Department of Dermatology, The Johns Hopkins Medical Institutions, Baltimore, Md (Drs Dugan and Watson); and the Section of Dermatology, Children's Hospital of Philadelphia, Department of Dermatology, University of Pennsylvania Medical Center (Drs Tunnessen and Honig). Dr Dugan is now with the National Cancer Institute, Bethesda, Md.

Footnotes
Accepted for publication June 11, 1992.

Reprint requests to Dermatology Branch, National Cancer Institute, Bldg 10, Room RM-12N238, National Institutes of Health, Bethesda, MD 20892 (Dr Dugan).

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