Background
The classic description of sclerema neonatorum (SN) is credited to Underwood, who described it in 1784 and appropriately termed it "skinbound disease." In 1817, Alibert introduced the term sclerema, derived from the Greek word skleros, meaning hard. Sclerema neonatorum is a disorder of the subcutaneous fat in debilitated neonates and is considered best as a sign of a potentially fatal underlying disease process and not a specific disease entity.[1] A thorough review of the nomenclature, clinical findings, histological features, differential diagnosis, and management of sclerema neonatorum was published in 2008.[2]
The Medscape Pediatric Dermatology Resource Center may be of interest.
Pathophysiology
In an infant, fat has a higher saturated-to-unsaturated fatty acid ratio compared to adult fat and thus, a higher melting point. Prematurity, hypothermia, shock, and metabolic abnormalities have been postulated to further increase this ratio, possibly as a result of enzymatic alteration allowing precipitation of fatty acid crystals within the lipocytes. This condition has been suggested to result in the dramatic clinical findings in affected skin. X-ray diffraction techniques have confirmed that infants with sclerema neonatorum have an increase in saturated fats and that the crystals within the fat cells are composed of triglycerides.[3]
Epidemiology
Frequency
United States
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.
International
A 10% incidence of sclerema neonatorum was documented in preterm neonates admitted to a hospital in Bangladesh. Risk factors for developing sclerema neonatorum in these infants included poor feeding, jaundice, and bacteremia.[4]
Mortality/Morbidity
Because sclerema neonatorum invariably is associated with serious underlying disease, the mortality rate is high. In different series, the reported mortality rates range from 67-88%, with death occurring hours to days after onset. If the underlying disease is treated successfully, the skin softens and returns to normal.
Race
No racial predilection has been reported.
Sex
Sclerema neonatorum shows a slight male predominance, with an estimated male-to-female ratio of 1.5:1.[5]
Age
Sclerema neonatorum is a disease confined to the newborn period. Sclerema neonatorum can present at birth, but onset within the first week of life is more common. The oldest reported infant presented with Pseudomonas septicemia at age 106 days.
Warwick WJ, Ruttenberg HD, Quie PG. Sclerema neonatorum--a sign, not a disease. JAMA. Jun 1 1963;184:680-3. [Medline].
Zeb A, Darmstadt GL. Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management. J Perinatol. Jul 2008;28(7):453-60. [Medline].
Kellum RE, Ray TL, Brown GR. Sclerema neonatorum. Report of a case and analysis of subcutaneous and epidermal-dermal lipids by chromatographic methods. Arch Dermatol. Apr 1968;97(4):372-80. [Medline].
Zeb A, Rosenberg RE, Ahmed NU, Saha SK, Chowdhury A, Ahmed S, et al. Risk factors for sclerema neonatorum in preterm neonates in Bangladesh. Pediatr Infect Dis J. May 2009;28(5):435-8. [Medline].
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Milunsky A, Levin SE. Sclerema neonatorum: a clinical study of 79 cases. S Afr Med J. Jul 2 1966;40(27):638-41. [Medline].
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Battin M, Harding J, Gunn A. Sclerema Neonatorum following hypothermia. J Paediatr Child Health. Oct 2002;38(5):533-4. [Medline].
Navarini-Meury S, Schneider J, Bührer C. Sclerema neonatorum after therapeutic whole-body hypothermia. Arch Dis Child Fetal Neonatal Ed. Jul 2007;92(4):F307. [Medline].
Torrelo A, Hernández A. Panniculitis in children. Dermatol Clin. Oct 2008;26(4):491-500. [Medline].
Sadana S, Mathur NB, Thakur A. Exchange transfusion in septic neonates with sclerema: effect on immunoglobulin and complement levels. Indian Pediatr. Jan 1997;34(1):20-5. [Medline].
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