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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.

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The classic description of this clinical entity is credited to Underwood, who described it in 1784 and appropriately termed it Skinbound Disease.

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A rare disorder limited to the newborn during the first weeks of life.

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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.

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Diffuse hardening of the subcutaneous adipose tissue in the newborn.

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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.

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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).

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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.

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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 6(3):474, 1987.  [PubMed: 3430253]

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