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At a glance

Rare disease characterized by firm, indurated, waxy skin lesions extending throughout the body, sparing the fat-free soles, palms, and genitalia. Temperature instability, restricted respiration, difficulty in feeding, and decreased spontaneous movement are characteristics. The prognosis is very poor.

History

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

Incidence

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

Diagnosis

Diffuse hardening of the subcutaneous adipose tissue in the newborn. The differential diagnosis in patients with subcutaneous fat necrosis, early scleroderma, Transient Hyperammonemia of the Newborn (THAN) is often difficult but of utmost importance because of the different prognosis of these illnesses.

Clinical aspects

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 neonatorum appears frequently in premature newborn, with associated diseases as sepsis, congenital heart disease, and respiratory distress. Sclerema neonatorum 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.

Precautions before anesthesia

Consider that it is a septic, usually dehydrated, newborn with incomplete diagnosis (high incidence of congenital heart failure). Check ammonia blood level, glucose, and 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).

Anesthetic considerations

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.

Pharmacological implications

Newborn with immature pathways, especially in the urea cycle.

Other condition to be considered

  • Subcutaneous fat necrosis of the newborn: Diseases of the subcutaneous adipose tissue with circumscribed hardening of skin on bony prominences with necrosis of adipocytes in subcutaneous tissue and a dense granulomatous infiltrate on histology. Self-limiting and lesions resolve within a few weeks to months.

References

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Heilbron  B, Saxe  N: Scleredema in an infant. Arch Dermatol[Archives of ...

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