Sclerema Neonatorum Clinical Presentation
- Author: India Mayo Hill, MD; Chief Editor: Dirk M Elston, MD more...
Half the infants affected by sclerema neonatorum are premature, and the others are full term but have a serious underlying illness. They are often of low birth weight (<2500 g) and have cyanosis and low Apgar score s. They are also more likely to be hypothermic and have lower protein levels in their serum. In one series, 75% of the mothers were healthy, while 25% had preeclampsia, placenta previa, or infection. Labor is usually normal, and delivery is spontaneous and nontraumatic.
Physical findings of sclerema neonatorum appear suddenly, first on the thighs and buttocks and then, spreading rapidly, often affecting all parts of the body except the palms, soles, and genitalia. The involved skin is pale, waxy, and firm to palpation. The skin cannot be pitted or pinched up because it is bound to underlying subcutaneous tissue, muscle, and bone. The affected infant often displays flexion contractures at the elbows, knees, and hips; temperature instability; restricted respiration; difficulty in feeding; and decreased spontaneous movement. Newborns may also present with masklike facies or “pseudotrismus,” an inability to completely open the mouth, secondary to the thickening of the skin over the face, arms, and hands.
Associated underlying conditions include pneumonia, septicemia, septic shock, hypothermia, metabolic acidosis, transient hyperammonemia of the newborn, respiratory distress syndrome, congenital heart defects, gastroenteritis, intestinal obstruction, and severe malnutrition.[2, 11, 9, 12] Two case reports have described sclerema neonatorum that developed after therapeutic hypothermia initiated for neonatal asphyxia.[13, 14]
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