Juvenile-onset hypergammaglobulinemic purpura and fetal congenital heart block

J Dermatol. 2006 Oct;33(10):714-8. doi: 10.1111/j.1346-8138.2006.00166.x.

Abstract

Waldenström's hypergammaglobulinemic purpura (HGP) is a rare chronic disorder characterized by recurrent purpura on the legs, a polyclonal increase in serum gamma-globulin, an elevated erythrocyte sedimentation rate and a positive rheumatoid factor. A 30-year-old primigravid woman with 14 years of HGP was found to have fetal bradycardia at 25 weeks' gestation. Laboratory investigations demonstrated positive anti-Ro/SSA and anti-La/SSB antibodies in the maternal serum. Cesarean delivery was performed at 39 weeks, and a 2750-g female infant was born with complete atrioventricular block. Fortunately, the neonatal period has been uneventful without need for pace-making. Maternal HGP exacerbated just after delivery, but resolved within 1 week without treatment. Physicians should be aware of the possible presence of neonatal lupus-related anti-Ro/SSA and anti-La/SSB autoantibodies in patients with HGP. Screening for these autoantibodies is important and could be used as a marker to identify and manage high-risk pregnancies.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Heart Block / congenital*
  • Heart Block / embryology
  • Humans
  • Infant, Newborn
  • Pregnancy
  • Pregnancy Complications, Hematologic*
  • Purpura, Hyperglobulinemic / complications*