PMID- 15234013
OWN - NLM
STAT- MEDLINE
DCOM- 20040917
LR  - 20220410
IS  - 0738-081X (Print)
IS  - 0738-081X (Linking)
VI  - 22
IP  - 2
DP  - 2004 Mar-Apr
TI  - Neonatal lupus erythematosus.
PG  - 125-8
AB  - Neonatal lupus Erythematosus (NLE) is a disorder characterized by maternal 
      autoantibodies against RNA protein complex, Ro/SSA or SSB/La. These maternal IgG 
      antibodies cross the placenta and potentially lead to fetal tissue damage and the 
      clinical manifestations NLE. NLE is uncommon, affects females more than males, 
      has no race predilection, and involves multiple organs. It has cutaneous 
      manifestations similar to subacute cutaneous lupus erythematosus (SCLE). In 
      addition to skin findings, patients with NLE have a significant risk of 
      congenital heart block (CHB), a potentially fatal complication. Less frequently, 
      hematologic and hepatic abnormalities occur. Approximately half of the reported 
      cases have skin disease and half have CHB. Approximately 10% have both CHB and 
      skin findings. The cutaneous, hematologic, and hepatic abnormalities are 
      transient, clearing by 6 months of age. However, CHB is permanent and requires a 
      pacemaker in many cases. The disorder results from the passive transfer of 
      maternal autoantibodies, anti-RoSSA and anti-La/SSB. Sontheimer and McCauliffe 
      reviewed the pathogenic role of anti-Ro antibody in NLE lesions and summarize 
      evidence supporting its pathogenic role. Additional evidence suggests the 
      possibility that Ro-antigen-specific T-cells may be present in SCLE patients and 
      have the capacity to cause direct injury to the skin. McCuistion and Schoch first 
      suggested this hypothesis in 1954 when they described an infant with LE skin 
      findings born to a mother with systemic lupus erythematosus (SLE). Since this 
      initial observation, a number of researchers have documented the role of maternal 
      autoantibodies in the pathogenesis of this disorder. The true incidence of NLE is 
      not known; however, it is known that NLE accounts for approximately 80% of all 
      cases of CHB, and the incidence of CHB is 1 per 20,000 live births. Therefore, it 
      can be assumed that the incidence of NLE is at least 1 per 12,500 live births.
FAU - Boh, Erin E
AU  - Boh EE
AD  - Department of Dermatology, Tulane University Health Sciences Center, New Orleans, 
      Louisiana 70112, USA. eboh@tulane.edu
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Clin Dermatol
JT  - Clinics in dermatology
JID - 8406412
SB  - IM
MH  - Female
MH  - Humans
MH  - Infant, Newborn
MH  - Infant, Newborn, Diseases/diagnosis/pathology
MH  - Lupus Erythematosus, Cutaneous/*diagnosis/pathology
MH  - Pregnancy
MH  - Pregnancy Complications/*diagnosis/pathology
RF  - 19
EDAT- 2004/07/06 05:00
MHDA- 2004/09/21 05:00
CRDT- 2004/07/06 05:00
PHST- 2004/07/06 05:00 [pubmed]
PHST- 2004/09/21 05:00 [medline]
PHST- 2004/07/06 05:00 [entrez]
AID - S0738081X03001391 [pii]
AID - 10.1016/j.clindermatol.2003.12.019 [doi]
PST - ppublish
SO  - Clin Dermatol. 2004 Mar-Apr;22(2):125-8. doi: 10.1016/j.clindermatol.2003.12.019.