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Congenital Generalized Myofibromatosis in a Neonate
Otobia G. Dimson, MD;
Beth A. Drolet, MD;
James F. Southern, MD;
Alice Rock, MD;
Andrea L. Winthrop, MD;
Nancy B. Esterly, MD
Arch Dermatol. 2000;136:597-600.
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REPORT OF A CASE
A 1-day-old female neonate developed signs of sepsis, bowel obstruction, and respiratory distress. She was born to a 34-year-old, gravida 2, para 1 woman at 40 weeks' gestation. The pregnancy was uncomplicated. The neonate was delivered vaginally and had lightly meconium-stained amniotic fluid. She weighed 3260 g and had Apgar scores of 8 and 9 at 1 and 5 minutes, respectively. The neonate was noted to have nodular lesions on the right neck and thighs, and several reddish purple macular lesions on the trunk and limbs (Figure 1). She had abdominal distention, but no hepatosplenomegaly or lymphadenopathy. Approximately 2 hours after delivery, the neonate developed respiratory distress. Radiographic studies revealed free air in the abdomen with a small-bowel perforation, multiple lytic lesions within the bones . . . [Full Text of this Article]
DIAGNOSTIC CHALLENGE
COMMENT
From the Departments of Dermatology (Drs Dimson, Drolet, and Esterly), Pediatrics (Dr Rock), Pediatric Surgery (Dr Winthrop), and Pathology (Dr Southern), Medical College of Wisconsin, Milwaukee.
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