Half of affected infants are premature. They are often
of low birth weight (<2500 g), cyanotic, and with low Apgar scores. Physical
findings appear suddenly, first on thighs and buttocks and spreading rapidly
to all parts of the body except hands, soles, and genitalia. The skin is firm
to hard and purplish in color. Skin cannot be pitted or picked up.
Temperature instability, restricted respiration, difficulty in feeding, and
decreased spontaneous movement are characteristics.
The classic description of this clinical entity is credited
to Underwood, who described it in 1784 and appropriately termed it
A rare disorder limited to the newborn during the first
weeks of life.
The differential diagnosis in patients with subcutaneous fat
necrosis, early scleroderma, Transient Hyperammonemia of the Newborn (THAN)
is often difficult but of almost importance because of the different
prognosis of these illnesses.
Diffuse hardening of the subcutaneous adipose tissue in
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
Consider that it is a septic,
usually dehydrated, newborn with incomplete diagnosis (high incidence of
congenital heart failure). Check ammonia blood level, glucose, urea. Check
temperature (they are less able than the normal newborn to keep
homeothermic). Consider long-term ventilatory assistance (the thickening of
the subcutaneous fat and the tense of the skin limit the excursion of the
rib cage and the abdomen).
Difficult intravenous access. Direct
laryngoscopy might be difficult because of reduced mouth opening (skin
limitation). Subjected to temperature instability during anesthesia,
especially severe hypothermia. All anesthetic considerations of anesthesia
for the premature must also be applied.
Newborn with immature pathways,
especially in the urea cycle.
Heilbron B, Saxe N: Scleredema in an infant. Arch Dermatol 122(12):1417, 1986.
Lindenberg JA, Milstein JM, Cox KL: Sclerema neonatorum: A sign of transient
hyperammonemia of the newborn. J Pediatr Gastroenterol Nutr