Sclerema Neonatorum Clinical Presentation

  • Author: Amy J Theos, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 11, 2010
 

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 scores.[6] 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 the underlying tissues. The affected infant often displays flexion contractures at the elbows, knees, and hips; temperature instability; restricted respiration; difficulty in feeding; and decreased spontaneous movement.

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Causes

Associated underlying conditions include pneumonia, septicemia, hypothermia, metabolic acidosis, respiratory distress syndrome, congenital heart defects, gastroenteritis, and intestinal obstruction.[7] Two case reports have described sclerema neonatorum that developed after therapeutic hypothermia initiated for neonatal asphyxia.[8, 9]

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

Craig A Elmets, MD  Professor and Chair, Department of Dermatology, Director, 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, and Society for Investigative Dermatology

Disclosure: Palomar Medical Technologies Stock None; Amgen Consulting fee Review panel membership; Astellas Consulting fee Review panel membership; Massachusetts Medical Society Salary Employment; Abbott Laboratories Grant/research funds Independent contractor

Specialty Editor Board

Daniel Mark Siegel, MD, MS  Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate

Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

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

Disclosure: Nothing to disclose.

References

  1. Warwick WJ, Ruttenberg HD, Quie PG. Sclerema neonatorum--a sign, not a disease. JAMA. Jun 1 1963;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. Jul 2008;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. Apr 1968;97(4):372-80. [Medline].

  4. 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].

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

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

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

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

  9. 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].

  10. Torrelo A, Hernández A. Panniculitis in children. Dermatol Clin. Oct 2008;26(4):491-500. [Medline].

  11. 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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