PMID- 8423406
OWN - NLM
STAT- MEDLINE
DCOM- 19930219
LR  - 20190723
IS  - 0022-202X (Print)
IS  - 0022-202X (Linking)
VI  - 100
IP  - 1
DP  - 1993 Jan
TI  - Neonatal lupus erythematosus.
PG  - 9S-13S
AB  - Neonatal lupus erythematosus (NLE) is an autoimmune disease whose major findings 
      are subacute cutaneous lupus erythematosus (SCLE) skin lesions and congenital 
      heart block. Babies have maternal anti-Ro/SSA, anti-La/SSB, or anti-U1RNP 
      autoantibodies. Anti-Ro/SSA are the predominant autoantibodies, having been found 
      in about 95% of cases. The autoantibodies pass through the placenta from mother 
      to child. Skin disease resolves at about the time that maternal autoantibodies 
      can no longer be detected in the baby. NLE therefore provides the strongest 
      clinical evidence that autoantibodies are involved in at least some 
      manifestations of lupus erythematosus, but there is as yet no definitive evidence 
      implicating autoantibodies in the disease process. Skin disease usually begins 
      after birth, is transient, and does not result in scarring. Cardiac disease 
      begins in utero, and the heart block is almost always permanent. Many babies 
      require pacemakers, and about 10% die from complications related to cardiac 
      disease. In some cases, transient liver disease or thrombocytopenia have been 
      observed. Individuals who had NLE usually have healthy childhoods but may develop 
      autoimmune disease in adulthood. Whether the later development of autoimmune 
      disease is a common or an unusual event is not yet known. Mothers of babies with 
      NLE may be asymptomatic initially, but with time usually develop symptoms of 
      autoimmune disease. The most typical constellation of symptoms in our group of 
      approximately 30 mothers of babies with NLE is that of Sjogren's syndrome. Most 
      babies exposed to anti-Ro/SSA autoantibodies during gestation will not develop 
      NLE. There is no test to determine prospectively which babies will be affected. 
      Treatment during gestation is still controversial and, if attempted, should be 
      reserved for fetuses with potentially life-threatening disease. Treatment after 
      birth consists of topical management for skin disease and pacemaker implantation, 
      if necessary, for heart block. Systemic steroids may be given for serious 
      internal disease.
FAU - Lee, L A
AU  - Lee LA
AD  - Department of Dermatology, University of Colorado School of Medicine, Denver.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - J Invest Dermatol
JT  - The Journal of investigative dermatology
JID - 0426720
SB  - IM
MH  - Adult
MH  - Autoimmune Diseases/immunology
MH  - Female
MH  - Heart Block/complications/congenital/therapy
MH  - Humans
MH  - Incidence
MH  - Infant, Newborn
MH  - *Lupus Erythematosus, Cutaneous/complications/immunology/therapy
MH  - Mothers
MH  - Myocardium/pathology
MH  - Pregnancy
MH  - Pregnancy Complications
MH  - Skin/pathology
RF  - 49
EDAT- 1993/01/01 00:00
MHDA- 1993/01/01 00:01
CRDT- 1993/01/01 00:00
PHST- 1993/01/01 00:00 [pubmed]
PHST- 1993/01/01 00:01 [medline]
PHST- 1993/01/01 00:00 [entrez]
AID - 0022-202X(93)92194-Y [pii]
AID - 10.1111/1523-1747.ep12355173 [doi]
PST - ppublish
SO  - J Invest Dermatol. 1993 Jan;100(1):9S-13S. doi: 10.1111/1523-1747.ep12355173.