Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • State of the Art
  • Published:

State-of-the-Art

Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management

Journal of Perinatology volume 28, pages 453–460 (2008)Cite this article

Abstract

Objective:

To review published literature on sclerema neonatorum (SN) in order to clarify its clinical presentation, histological features and management compared with two other diseases: subcutaneous fat necrosis of the newborn (SCFN) and scleredema.

Study Design:

PubMed database was searched using the key words Sclerema neonatorum. A total of 55 articles from peer-reviewed journals were reviewed and summarized.

Result:

SN, SCFN and scleredema are diseases of the subcutaneous adipose tissue. SN is characterized by hardening of the skin that gets bound down to the underlying muscle and bone, hindering respiration and feeding and is associated with congenital anomalies, cyanosis, respiratory illnesses and sepsis. Histology of the skin biopsy shows thickening of the trabeculae supporting the subcutaneous adipose tissue and a sparse inflammatory infiltrate of lymphocytes, histiocytes and multinucleate giant cells. SCFN has circumscribed hardening of skin on bony prominences with necrosis of adipocytes in subcutaneous tissue and a dense granulomatous infiltrate on histology. Scleredema is characterized by hardening of the skin along with edema; histology shows inflammatory infiltrate and edema in skin and subcutaneous tissues. SN has a high case fatality rate whereas SCFN and scleredema are self-limiting and lesions resolve within a few weeks to months. Exchange transfusion may improve survival in SN.

Conclusion:

The histological features of skin biopsy should be used to establish diagnosis of SN, SCFN and scleredema as disease-specific treatment is imperative in SN due to high fatality.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1
Figure 2
Figure 3

References

  1. Requena L . Normal subcutaneous fat, necrosis of adipocytes and classification of the panniculitides. Semin Cutan Med Surg 2007; 26 (2): 66–70.

    Article  CAS  Google Scholar 

  2. Requena L, Sanchez Yus E . Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 2001; 45 (3): 325–361.

    Article  CAS  Google Scholar 

  3. Villacorte G, Frank DJ . Sclerema neonatorum. A report of nine cases. Ohio State Med J 1967; 63 (1): 57–59.

    CAS  PubMed  Google Scholar 

  4. Warwick WJ, Ruttenberg HD, Quie PG . Sclerema neonatorum—a sign, not a disease. JAMA 1963; 184: 680–683.

    Article  CAS  Google Scholar 

  5. Behrman RE, Kliegman RM, Jenson HB . Nelson Textbook of Pediatrics,17th edn. Saunders: USA, 2004,pp 2211–2212.

    Google Scholar 

  6. Joncas J . Sclerema neonatorum: report of an unusual case. Can Med Assoc J 1959; 80 (5): 365–368.

    CAS  PubMed  PubMed Central  Google Scholar 

  7. Djojodiguno ST . Sclerema neonatorum (literature review). Paediatr Indones 1965; 5 (1–2): 28–34.

    CAS  PubMed  Google Scholar 

  8. Elliott RI . Sclerema. Proc R Soc Med 1959; 52: 1018–1021.

    CAS  PubMed  PubMed Central  Google Scholar 

  9. Fretzin DF, Arias AM . Sclerema neonatorum and subcutaneous fat necrosis of the newborn. Pediatr Dermatol 1987; 4 (2): 112–122.

    Article  CAS  Google Scholar 

  10. Horsfield GI, Yardley HJ . Sclerema neonatorum. J Invest Dermatol 1965; 44: 326–332.

    Article  CAS  Google Scholar 

  11. Brain RT . Sclerema neonatorum. Br J Dermatol 1953; 65 (10): 373–374.

    Article  CAS  Google Scholar 

  12. Ghosal SP, Nag Chaudhuri J . Sclerematous skin changes in infantile diarrhoea. Indian J Pediatr 1961; 28: 1–10.

    Article  CAS  Google Scholar 

  13. Navarini-Meury S, Schneider J, Buhrer C . Sclerema neonatorum after therapeutic whole-body hypothermia. Arch Dis Child Fetal Neonatal Ed 2007; 92 (4): F307.

    Article  Google Scholar 

  14. Hughes WE, Hammond ML . Sclerema neonatorum. J Pediatr 1948; 32 (6): 676–692.

    Article  CAS  Google Scholar 

  15. Khetarpal SK, Subrahmanyam VV . Sclerema neonatorum: a study of 17 cases. Indian J Pediatr 1964; 31: 8–13.

    Article  CAS  Google Scholar 

  16. Black MM . Panniculitis. J Cutan Pathol 1985; 12 (3–4): 366–380.

    Article  CAS  Google Scholar 

  17. 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 (4): 372–380.

    Article  CAS  Google Scholar 

  18. Proks C, Valvoda V . Fatty crystals in sclerema neonatorum. J Clin Pathol 1966; 19 (2): 193–195.

    Article  CAS  Google Scholar 

  19. Pasyk K . Sclerema neonatorum. Light and electron microscopic studies. Virchows Arch A Pathol Anat Histol 1980; 388 (1): 87–103.

    Article  CAS  Google Scholar 

  20. Channon HJ, Harrison GA . The chemical nature of the subcutaneous fat in the normal and sclerematous infant. Biochem J 1926; 20 (1): 84–92.

    Article  CAS  Google Scholar 

  21. Jakovcic S . Lipid metabolism in the developing fetus and the newborn. Pediatr Clin North Am 1965; 12: 585–594.

    Article  CAS  Google Scholar 

  22. 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 (1): 50–55.

    CAS  PubMed  Google Scholar 

  23. Levin SE, Bakst CM, Isserow L . Sclerema neonatorum treated with corticosteroids. Br Med J 1961; 2 (5266): 1533–1536.

    Article  CAS  Google Scholar 

  24. Marks MB . Subcutaneous adipose derangements of the newborn. Am J Dis Child 1962; 104: 122–130.

    CAS  PubMed  Google Scholar 

  25. Sadana S, Mathur NB, Thakur A . Exchange transfusion in septic neonates with sclerema: effect on immunoglobulin and complement levels. Indian Pediatr 1997; 34 (1): 20–25.

    CAS  PubMed  Google Scholar 

  26. 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 1982; 49 (399): 519–523.

    Article  CAS  Google Scholar 

  27. Vain NE, Mazlumian JR, Swarner OW, Cha CC . Role of exchange transfusion in the treatment of severe septicemia. Pediatrics 1980; 66 (5): 693–697.

    CAS  PubMed  Google Scholar 

  28. Xanthou M, Xypolyta A, Anagnostakis D, Economou-Mavrou C, Matsaniotis N . Exchange transfusion in severe neonatal infection with sclerema. Arch Dis Child 1975; 50 (11): 901–902.

    Article  CAS  Google Scholar 

  29. Prod'hom LS, Choffat JM, Frenck N, Mazoumi M, Relier JP, Torrado A . Care of the seriously ill neonate with hyaline membrane disease and with sepsis (sclerema neonatorum). Pediatrics 1974; 53 (2): 170–181.

    CAS  PubMed  Google Scholar 

  30. Silverman AK . Panniculitis in infants. Arch Dermatol 1985; 121 (7): 834.

    Article  CAS  Google Scholar 

  31. Dunn PM . Dr Charles West (1816–98) of London and the cold syndrome. Arch Dis Child 1991; 66 (4 Spec No): 455–456.

    Article  CAS  Google Scholar 

  32. Kumar R, Agarwal PK, Wakhlu AK, Wakhlu I . Scleredema in a 6-week-old baby. Indian Pediatr 1991; 28 (10): 1195–1197.

    CAS  PubMed  Google Scholar 

  33. Beers WH, Ince A, Moore TL . Scleredema adultorum of Buschke: a case report and review of the literature. Semin Arthritis Rheum 2006; 35 (6): 355–359.

    Article  Google Scholar 

  34. Milunsky A, Levin SE . Sclerema neonatorum: a clinical study of 79 cases. S Afr Med J 1966; 40 (27): 638–641.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of International Health, International Center for Advancing Neonatal Health, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD, USA

    A Zeb & G L Darmstadt

Authors
  1. A Zeb
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. G L Darmstadt
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to G L Darmstadt.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Zeb, A., Darmstadt, G. Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management. J Perinatol 28, 453–460 (2008). https://doi.org/10.1038/jp.2008.33

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/jp.2008.33

Keywords

This article is cited by

Search

Advanced search

Quick links