Sclerema neonatorum generally presents in gravely
ill, often preterm, infants with diffuse yellowish-white woody induration.
The affected skin is cold, nonpitting, and immobile, given an appearance of
tight, shiny, bound-down skin with herniation of the usually spared nipple
area. Beginning on the buttocks, thighs, or calves, the process extends
rapidly and symmetrically to involve nearly the entire body surface.
Sclerema appear frequently in premature newborn, with associated diseases as
sepsis, congenital heart disease, respiratory distress. Sclerema is not
specific of THAN, but an observation has been made that sclerema neonatorum
may be an early manifestation of THAN; consequently, it is recommended that
the blood ammonia concentration be determined in any infant with this
clinical sign. THAN infants have an excellent chance of survival, if
recognized early.