Are You Confident of the Diagnosis?
Characteristic findings on physical examination
Sclerema neonatorum (SN) is a very rare disorder of subcutaneous fat that manifests as broad firm areas of hard skin within the first few days after birth (rarely later in the neonatal period) and generally progresses to involve nearly all the subcutaneous and even possibly the visceral fat.
The clinical setting of a preterm (rarely term) neonate who is septic, hypotensive, dehydrated or has some other severe medical or surgical stress is characteristic. The hardening progresses to involve the majority of the body except palms, soles and genitalia (these areas have less fat). SN is seen less in areas with access to advanced neonatal care due to better management of severe neonatal illness.
Expected results of diagnostic studies
Histopathology of SN is a non-inflammatory thickening of the septae within the fat and needle-shaped clefts arranged radially within adipocytes. The changes may be subtle and the lack of inflammation despite the severe clinical findings is characteristic.
Subcutaneous fat necrosis of the newborn (SCFN) is more localized and occurs in healthy (typically full term) infants.
Stiff skin syndrome is a slowly progressive fibrosis of the skin but the neonates are less ill than in SN.
Scleredema is an inflammatory process that causes thickening that is less extensive than SN. Scleredema histopathology shows an inflammatory reaction in the dermis with fibrosis, and the prognosis is generally good.
Congenital lymphedema is softer and doughier feeling and not as progressive as SN.
Cellulitis and necrotizing fasciitis are more localized with erythematous or purpuric skin changes and less fibrosis.
Who is at Risk for Developing this Disease?
SN is a disease of severely ill neonates. Sepsis, shock or surgical stress are most commonly associated. Illnesses that have been described in association with SN include necrotizing enterocolitis, intracranial hemorrhage, pneumonia. SN is likely due to abnormal fat conversion from saturated to unsaturated fats in the setting of severe neonatal illness and occurs nearly exclusively in preterm infants.
Many of the recent reports are from developing countries as sclerema is becoming exceedingly rare in developed countries.
A recent study from Bangladesh noted risk factors of low maternal education, neonatal jaundice and poor feeding.
What is the Cause of the Disease?
SN is likely a final common pathway of many different types of severe stresses leading to crystal formation and solidification of the saturated fats more prevalent in newborns. This may be due to circulatory insufficiency, cold temperature or an enzymatic process.
Systemic Implications and Complications
The extensive bound down subcutaneous tissue causes restriction of breathing and feeding. The enzymatic effects on the fat can lead to hypoglycemia, hypercalcemia and other electrolyte imbalances. The death rate is over 50% and in some series over 80%, but if recovery is successful the skin will return to normal.
Treatment is supportive. Monitor fluid status, electrolytes, evaluate for sources of sepsis or other stress. Survival is uncommon. Treatment with systemic steroids and therapeutic warming has not improved survival. Exchange transfusion has been used with some success, especially in India.
Optimal Therapeutic Approach for this Disease
Finding the source of the sepsis or other insult is key, and then supportive care is indicated.
An evaluation for sepsis, shock or surgical stress is essential as these are the most commonly associated potential precipitants of the process. Illnesses that have been described in association with SN include necrotizing enterocolitis, intracranial hemorrhage, pneumonia, hypoglycemia, hypercalcemia and other electrolyte imbalances. Treatment is supportive, with monitoring fluid status, electrolytes, and pulmonary function.
Unusual Clinical Scenarios to Consider in Patient Management
SN is less prevalent in developed countries likely due to both more advanced neonatal care as well as more available diagnostic procedures to help differentiate SN from SCFN and scleredema.
What is the Evidence?
Narayanan, I, Mitter, A, Gujral, VV. “A comparative study on the value of exchange and blood transfusion in the management of severe neonatal septicemia with sclerema”. Indian J Pediatr. vol. 49. 1982. pp. 519-23. (Exchange transfusion appears to have some role in therapy.)
Sadana, S, Mathur, NB, Thakur, A. “Exchange transfusion in septic neonates with sclerema: effect on immunoglobulin and complement levels”. Indian Pediatrics. vol. 34. 1997. pp. 20-5. (Exchange transfusion appears to have some role in therapy.)
Zeb, A, Rosenberg, RE, Ahmed, NU, Saha, SK, Chowdhury, RE, Ahmed, S, Darmstadt, GL. “Risk factors for sclerema neonatorum in preterm neonates in Bangladesh”. Pediatr Infect Dis J. vol. 28. 2009. pp. 435-8. (Jaundice, poor feeding and low maternal education were associated with sclerema and most patients had associated gram negative sepsis.)
Warwick, WJ, Ruttenberg, HD, Quie, PG. “Sclerema neonatorum-a sign, not a disease”. JAMA.. vol. 184. 1963. pp. 680-683. (One of the original articles explaining that sclerema may be a final clinical pathway of many types of severe neonatal illness such as sepsis.)
Levin, SE, Bakst, CM, Isserow, L. “Sclerema neonatorum treated with corticosteroids”. Br Med J. vol. 2. 1961. pp. 1533-6. (Corticosteroids may be a treatment option.)
Zeb, A, Darmstadt, GL. “Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management”. J Perinatol. vol. 28. 2008. pp. 453-60. (This is an excellent review of the disease and how it is separated from other panniculititides and fibrotic skin disorders.)
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