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Sclerema neonatorum

Author
Raegan Hunt, MD, PhD
Section Editor
Moise L Levy, MD
Deputy Editor
Rosamaria Corona, MD, DSc

INTRODUCTION

Sclerema neonatorum is an uncommon severe panniculitis that manifests as a diffuse skin hardening in critically ill, premature, and low-birthweight infants [1]. The hardened skin and subcutaneous fat become bound down and adherent to underlying muscle and bone, such that the basic functions of breathing, feeding, and movement are restricted. Affected infants suffer from comorbid illnesses, such as sepsis, dehydration, severe respiratory or gastrointestinal disease, and congenital malformations. Mortality is high; however, with current standards of neonatal intensive care, sclerema neonatorum is thought to be exceptionally uncommon. Regardless, cases of sclerema neonatorum occurring in neonatal intensive care settings continue to be reported [2], and it is important that clinicians are able to diagnose and treat this entity.

This topic discusses the pathogenesis, clinical manifestations, diagnosis, and management of sclerema neonatorum.

EPIDEMIOLOGY

Sclerema neonatorum characteristically affects newborn infants and typically develops within the first week of life, although a few cases have been reported to occur beyond the neonatal period. Aggregation of case reports suggests that males may be affected slightly more often than females (male to female ratio, 1.6:1) [1]. Maternal parity does not appear to be a risk factor [3].

The incidence of sclerema neonatorum is not known. The largest case series have been published between 1940 and 1970, while fewer cases have been reported in last few decades [4]. It has been postulated that improved perinatal intensive care has substantially reduced the number of affected infants, rendering sclerema neonatorum a rare diagnosis in the setting of modern neonatal intensive care [5]. Limited data suggest that the incidence of sclerema neonatorum may be higher in areas with less access to high acuity neonatal care. A study evaluating premature newborns at a tertiary pediatric hospital in Bangladesh from 1998 to 2003 reported a 10 percent incidence of sclerema neonatorum [6].

PATHOGENESIS

The pathogenesis of sclerema neonatorum remains unknown. Subcutaneous adipose tissue in neonates is enriched in saturated fats as compared with the subcutaneous fat composition of older people. This special biochemical property of neonatal fat makes it more likely to harden in a cold environment. It has been suggested that decreased body temperatures encountered in clinical shock trigger subcutaneous adipose hardening in sclerema neonatorum [7]. However, fat hardening should not occur until skin temperature is below the freezing point, which argues against this explanation. Additional theories propose that sclerema neonatorum is a consequence of abnormal fat metabolism, results from dysfunction of the connective tissue surrounding the adipocytes, or is a downstream effect triggered by systemic toxicity [8-10].

       

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Literature review current through: Nov 2016. | This topic last updated: Thu Nov 03 00:00:00 GMT 2016.
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