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Juvenile‐onset hypergammaglobulinemic purpura and fetal congenital heart block
Author(s) -
MAEDATANAKA Maki,
HARUTA Shoji,
SADO Toshiyuki,
UCHIDA Yumiko,
TAKAHASHI Yukihiro,
ASADA Hideo,
MIYAGAWA Sachiko
Publication year - 2006
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2006.00166.x
Subject(s) - medicine , gestation , rheumatoid factor , heart block , pregnancy , pediatrics , autoantibody , obstetrics , antibody , immunology , arthritis , electrocardiography , genetics , biology
Waldenström's hypergammaglobulinemic purpura (HGP) is a rare chronic disorder characterized by recurrent purpura on the legs, a polyclonal increase in serum γ‐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.