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.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version September 2024
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Neonatal lupus erythematosus?
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