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Neonatal lupus erythematosus

Disease definition

A rare systemic autoimmune disease characterized by cutaneous lesions, hepatic dysfunction, hematological abnormalities, and/or cardiac arrhythmia, and caused by transplacental passage of maternal SS-A and SS-B autoantibodies. The most typical cutaneous manifestation is a macular annular erythema affecting the head, but also trunk and extremities. Other reversible features include anemia, neutropenia, thrombocytopenia, and elevation of liver parameters with hepatomegaly. The most severe presentation of the disease is irreversible congenital total atrioventricular block.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: Neonatal
  • ICD-10: M32.8
  • ICD-11: KA07.0
  • OMIM: -
  • UMLS: C0409979
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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