- Raegan Hunt, MD, PhD
Raegan Hunt, MD, PhD
- Assistant Professor of Dermatology and Pediatrics
- Texas Children's Hospital
- Baylor College of Medicine
Sclerema neonatorum is an uncommon severe panniculitis that manifests as a diffuse skin hardening in critically ill, premature, and low-birthweight infants . 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 , 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. Subcutaneous fat necrosis of the newborn is discussed separately. (See "Subcutaneous fat necrosis of the newborn".)
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) . Maternal parity does not appear to be a risk factor .
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 . 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 . 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 .
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 . 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].To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Zeb A, Darmstadt GL. Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management. J Perinatol 2008; 28:453.
- Spohn GP, Pietras TA, Stone MS. Delayed-Onset Sclerema Neonatorum in a Critically Ill Premature Infant. Pediatr Dermatol 2016; 33:e168.
- KHETARPAL SK, SUBRAHMANYAM VV. SCLEREMA NEONATORUM: A STUDY OF 17 CASES. Indian J Pediatr 1964; 31:8.
- Polcari IC, Stein SL. Panniculitis in childhood. Dermatol Ther 2010; 23:356.
- Weedon D. Panniculitis. In: Weedon's Skin Pathology, 3rd ed, Weedon D (Ed), Churchill Livingstone/Elsevier, Edinburgh 2010. p.1041.
- Zeb A, Rosenberg RE, Ahmed NU, et al. Risk factors for sclerema neonatorum in preterm neonates in Bangladesh. Pediatr Infect Dis J 2009; 28:435.
- HUGHES WE, HAMMOND ML. Sclerema neonatorum. J Pediatr 1948; 32:676.
- 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 1968; 97:372.
- WARWICK WJ, RUTTENBERG HD, QUIE PG. Sclerema neonatorum--a sign, not a disease. JAMA 1963; 184:680.
- Villacorte G, Frank DJ. Sclerema neonatorum. A report of nine cases. Ohio State Med J 1967; 63:57.
- Christiansen SD, Desai NS, Pulito AR, Slack MR. Ischemic extremities due to compartment syndromes in a septic neonate. J Pediatr Surg 1983; 18:641.
- Requena L, Yus ES. Panniculitis. Part I. Mostly septal panniculitis. J Am Acad Dermatol 2001; 45:163.
- Rapini RP. Practical dermatopathology, Rapini RP (Ed), Elsevier Mosby, Philadelphia 2005.
- Hogeling M, Meddles K, Berk DR, et al. Extensive subcutaneous fat necrosis of the newborn associated with therapeutic hypothermia. Pediatr Dermatol 2012; 29:59.
- Del Pozzo-Magaña BR, Ho N. Subcutaneous Fat Necrosis of the Newborn: A 20-Year Retrospective Study. Pediatr Dermatol 2016; 33:e353.
- Markus JR, de Carvalho VO, Abagge KT, Percicotte L. Ice age: a case of cold panniculitis. Arch Dis Child Fetal Neonatal Ed 2011; 96:F200.
- Heilbron B, Saxe N. Scleredema in an infant. Arch Dermatol 1986; 122:1417.
- Kumar R, Agarwal PK, Wakhlu AK, Wakhlu I. Scleredema in a 6-week-old baby. Indian Pediatr 1991; 28:1195.
- Smitt JH, van Asperen CJ, Niessen CM, et al. Restrictive dermopathy. Report of 12 cases. Dutch Task Force on Genodermatology. Arch Dermatol 1998; 134:577.
- Navarro CL, Esteves-Vieira V, Courrier S, et al. New ZMPSTE24 (FACE1) mutations in patients affected with restrictive dermopathy or related progeroid syndromes and mutation update. Eur J Hum Genet 2014; 22:1002.
- McKenna T, Sola Carvajal A, Eriksson M. Skin Disease in Laminopathy-Associated Premature Aging. J Invest Dermatol 2015; 135:2577.
- Darragh CT, Eichel Y, Lewis FJ, et al. Sclerodermatous skin changes in an infant. Pediatr Dermatol 2014; 31:387.
- Gonzalo S, Kreienkamp R. DNA repair defects and genome instability in Hutchinson-Gilford Progeria Syndrome. Curr Opin Cell Biol 2015; 34:75.
- Bwibo NO, Anderson BT. Sclerema neonatorum (a study of 16 cases in the special care unit, Mulago Hospital, Kampala). East Afr Med J 1970; 47:50.
- LEVIN SE, BAKST CM, ISSEROW L. Sclerema neonatorum treated with corticosteroids. Br Med J 1961; 2:1533.
- Milunsky A, Levin SE. Sclerema neonatorum: a clinical study of 79 cases. S Afr Med J 1966; 40:638.
- Sadana S, Mathur NB, Thakur A. Exchange transfusion in septic neonates with sclerema: effect on immunoglobulin and complement levels. Indian Pediatr 1997; 34:20.
- Xanthou M, Xypolyta A, Anagnostakis D, et al. Exchange transfusion in severe neonatal infection with sclerema. Arch Dis Child 1975; 50:901.
- Vain NE, Mazlumian JR, Swarner OW, Cha CC. Role of exchange transfusion in the treatment of severe septicemia. Pediatrics 1980; 66:693.
- Buster KJ, Burford HN, Stewart FA, et al. Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments. Cutis 2013; 92:83.