Description, Causes and Risk Factors:
Sclerema appearing at birth or in early infancy, usually in premature and hypothermic infants, as sharply demarcated and yellowish white indurated plaques that usually involve the cheeks, buttocks, shoulders, and calves; subcutaneous fat has a high proportion of saturated fatty acids; microscopically, there is thickening of interlobular fibrous tissue and formation of triglyceride crystals and foreign body giant cells; prognosis is poor for widespread lesions, but localized lesions may resolve slowly over a period of many months.
Sclerema neonatorum is an uncommon disease characterized by a diffuse, rapidly spreading, non-edematous, tallow-like hardening of the subcutaneous tissue of infants in the first week or so of life. The skin in the involved areas cannot be picked up and the subcutaneous tissue seems bound down to subjacent muscle and bone. The involvement usually starts on the buttocks, thighs or the trunk but may spread to involve any area of the body except the soles, palms and genitalia. The affected infants often have other associated diseases, e.g., pneumonitis, enteritis, intracranial haemorrhage or congenital heart disease, while in 5 of the reported cases, the mothers were ill during pregnancy, thus accounting for the fact that the majority of infants affected are debilitated or feeble at birth.
Almost invariably these infants show marked difficulty with respect to temperature control and little tendency to spontaneous movement, especially when large areas are involved. The disease is usually present a few days after birth, the average age of onset being about 4 days, but it might be present at birth or may only be seen several weeks later.
Dehydration, weakness, low body temperature and acidosis have all been considered as etiological factors. Several theories have been proposed including low oleic acid content of fat plus a low body temperature. Shock and peripheral circulatory insufficiency as precipitating factors have recently received considerable attention. These factors may result from normal obstetrical trauma when condition is present at birth or from precding or concomitant disease to which these infants are unusually predisposed, when the manifestation appear later. The alteration in and thickening of the collagen fibres which is the essential histopathological lesion is considered to be due to a disturbance of cell metabolism consequent the peripheral circulatory failure.
Finally, infection and trauma have been suggested as possible etiological factors; whereas they play an important role in subcutaneous fat necrosis, there is no clinical or pathological evidence that they have any influence in sclerema neonatrum.
The exact incidence of sclerema neonatorum is unknown. All studies describe sclerema neonatorum as extremely rare. The number of reported cases in recent years has declined, probably as a result of better neonatal care. The prognosis is poor, the clinical course, with few exceptions, being rapidly downhill with a fatal termination only a few days after the appearance of the manifestations.
When the disease makes its appearance in infancy itruns a fairly acute and steadily progressive course, butmay however become chronic when it is subjected toperiods of arrest, giving rise later on to areas of baldness,facial hemiatrophy, discrepancy in limb proportions,muscle contractures and anomalies of pigmentation. Whenthe fingers and toes are affected it is called sclerodactyly.
Laboratory abnormalities associated with sclerema neonatorum correlate with the underlying disease process. Hypoglycemia, metabolic acidosis, respiratory alkalosis, hyperkalemia, hypocalcemia, and elevated blood urea are common, albeit nonspecific, findings. A complete sepsis workup should be initiated in all infants with sclerema neonatorum.
Recognition and the prompt institution of therapy specific for the underlying disease are mandatory. Careful monitoring, correction of electrolyte abnormalities, respiratory support, correction of hypovolemia, and control of hypothermia are important in sclerema neonatorum patients.
Treatment Options May Include:
Systemic steroids: The value of systemic steroids is controversial. No controlled studies have demonstrated improved survival with the use of systemic steroids in sclerema neonatorum, although they are often used.
Exchange transfusions: A randomized controlled trial demonstrated a significant survival benefit in septic neonates with sclerema neonatorum who were treated with exchange transfusion.
Antibiotics: Some authors advocate the prompt institution of prophylactic broad-spectrum antibiotic therapy for possible associated sepsis.
NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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