Sclerema Neonatorum Clinical Presentation

  • Author: India Mayo Hill, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Apr 07, 2016
 

History

Half the infants affected by sclerema neonatorum are premature, and the others are full term but have a serious underlying illness. They are often of low birth weight (<2500 g) and have cyanosis and low Apgar score s.[8] They are also more likely to be hypothermic and have lower protein levels in their serum.[9] In one series, 75% of the mothers were healthy, while 25% had preeclampsia, placenta previa, or infection. Labor is usually normal, and delivery is spontaneous and nontraumatic.

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Physical

Physical findings of sclerema neonatorum appear suddenly, first on the thighs and buttocks and then, spreading rapidly, often affecting all parts of the body except the palms, soles, and genitalia. The involved skin is pale, waxy, and firm to palpation. The skin cannot be pitted or pinched up because it is bound to underlying subcutaneous tissue, muscle, and bone. The affected infant often displays flexion contractures at the elbows, knees, and hips; temperature instability; restricted respiration; difficulty in feeding; and decreased spontaneous movement. Newborns may also present with masklike facies or “pseudotrismus,” an inability to completely open the mouth, secondary to the thickening of the skin over the face, arms, and hands.[10]

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Causes

Associated underlying conditions include pneumonia, septicemia, septic shock, hypothermia, metabolic acidosis, transient hyperammonemia of the newborn, respiratory distress syndrome, congenital heart defects, gastroenteritis, intestinal obstruction, and severe malnutrition.[2, 11, 9, 12] Two case reports have described sclerema neonatorum that developed after therapeutic hypothermia initiated for neonatal asphyxia.[13, 14]

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Contributor Information and Disclosures
Author

India Mayo Hill, MD Resident Physician, Department of Dermatology, University of Alabama at Birmingham School of Medicine

India Mayo Hill, MD is a member of the following medical societies: Alabama Dermatology Society, American Academy of Dermatology, American Society for Dermatologic Surgery, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Craig A Elmets, MD Professor and Chair, Department of Dermatology, Director, Chemoprevention Program Director, Comprehensive Cancer Center, UAB Skin Diseases Research Center, University of Alabama at Birmingham School of Medicine

Craig A Elmets, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Immunologists, American College of Physicians, American Federation for Medical Research, Society for Investigative Dermatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: University of Alabama at Birmingham; University of Alabama Health Services Foundation<br/>Serve(d) as a speaker or a member of a speakers bureau for: Ferndale Laboratories<br/>Received research grant from: NIH, Veterans Administration, California Grape Assn<br/>Received consulting fee from Astellas for review panel membership; Received salary from Massachusetts Medical Society for employment; Received salary from UpToDate for employment. for: Astellas.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Daniel Mark Siegel, MD, MS Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate Medical Center

Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Association for Physician Leadership, American Society for Dermatologic Surgery, American Society for MOHS Surgery, International Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Acknowledgements

Gloria H Nguyen, MD Cutaneous Oncology Research Fellow, Department of Dermatology, University of Alabama at Birmingham School of Medicine

Gloria H Nguyen, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Amy J Theos, MD Director of Pediatric Dermatology, Associate Professor, Department of Dermatology, University of Alabama at Birmingham

Amy J Theos, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

References
  1. Warwick WJ, Ruttenberg HD, Quie PG. Sclerema neonatorum--a sign, not a disease. JAMA. 1963 Jun 1. 184:680-3. [Medline].

  2. Zeb A, Darmstadt GL. Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management. J Perinatol. 2008 Jul. 28(7):453-60. [Medline].

  3. 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 Apr. 97(4):372-80. [Medline].

  4. Breukhoven PE, Kerkhof GF, Willemsen RH, Hokken-Koelega AC. Fat mass and lipid profile in young adults born preterm. J Clin Endocrinol Metab. 2012 Apr. 97(4):1294-302. [Medline].

  5. 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. 2009 May. 28(5):435-8. [Medline].

  6. Bwibo NO, Anderson BT. Sclerema neonatorum (a study of 16 cases in the special care unit, Mulago Hospital, Kampala). East Afr Med J. 1970 Jan. 47(1):50-5. [Medline].

  7. Afroze F, Pietroni MA, Chisti MJ. Recurrent sclerema in a young infant presenting with severe sepsis and severe pneumonia: an uncommon but extremely life-threatening condition. J Health Popul Nutr. 2013 Dec. 31:538-42. [Medline]. [Full Text].

  8. Milunsky A, Levin SE. Sclerema neonatorum: a clinical study of 79 cases. S Afr Med J. 1966 Jul 2. 40(27):638-41. [Medline].

  9. Chisti MJ, Saha S, Roy CN, et al. Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka. Ann Trop Paediatr. 2009 Mar. 29(1):45-50. [Medline].

  10. Taeusch, HW, Ballard RA, Gleason CA, Avery ME. Avery's Diseases of the Newborn. 8th ed. Philadelphia, PA: Elsevier Saunders; 2005. 1516-8.

  11. Fretzin DF, Arias AM. Sclerema neonatorum and subcutaneous fat necrosis of the newborn. Pediatr Dermatol. 1987 Aug. 4(2):112-22. [Medline].

  12. Lindenberg JA, Milstein JM, Cox KL. Sclerema neonatorum: a sign of transient hyperammonemia of the newborn. J Pediatr Gastroenterol Nutr. 1987 May-Jun. 6(3):474-6. [Medline].

  13. Battin M, Harding J, Gunn A. Sclerema Neonatorum following hypothermia. J Paediatr Child Health. 2002 Oct. 38(5):533-4. [Medline].

  14. Navarini-Meury S, Schneider J, Bührer C. Sclerema neonatorum after therapeutic whole-body hypothermia. Arch Dis Child Fetal Neonatal Ed. 2007 Jul. 92(4):F307. [Medline].

  15. Polcari IC, Stein SL. Panniculitis in childhood. Dermatol Ther. 2010 Jul-Aug. 23(4):356-67. [Medline].

  16. Sadana S, Mathur NB, Thakur A. Exchange transfusion in septic neonates with sclerema: effect on immunoglobulin and complement levels. Indian Pediatr. 1997 Jan. 34(1):20-5. [Medline].

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