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Sclerema neonatorum

MedGen UID:
20677
•Concept ID:
C0036415
•
Congenital Abnormality
Synonym: Sclerema Neonatorum
SNOMED CT: Sclerema neonatorum (206539008); Sclerema adiposum (206539008); Underwood's disease (206539008); Pre-agonal induration (206539008)
 
Monarch Initiative: MONDO:0043783

Definition

A diffuse hardening of skin and subcutaneous adipose tissue, associated with minimal inflammation without fat necrosis, that typically affects critically ill preterm neonates during the first week of life. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSclerema neonatorum

Professional guidelines

PubMed

Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management.
Zeb A, Darmstadt GL
J Perinatol 2008 Jul;28(7):453-60. Epub 2008 Mar 27 doi: 10.1038/jp.2008.33. PMID: 18368059
Effects of Flos Carthami Co injection in the treatment of sclerema neonatorum.
Gong CF, Yu PL
J Tradit Chin Med 1983 Jun;3(2):141-3. PMID: 6555449
Effect of fresh blood transfusion in the treatment of sclerema neonatorum.
Sarwono E, Marseno FX, Kwari Satjadibrata R, Polanunu AR
Paediatr Indones 1973 Apr;13(4):120-33. PMID: 4784026

Recent clinical studies

Etiology

Panniculitis in children.
Grassi S, Borroni RG, Brazzelli V
G Ital Dermatol Venereol 2013 Aug;148(4):371-85. PMID: 23900159
Risk factors for sclerema neonatorum in preterm neonates in Bangladesh.
Zeb A, Rosenberg RE, Ahmed NU, Saha SK, Chowdhury A, Ahmed S, Darmstadt GL
Pediatr Infect Dis J 2009 May;28(5):435-8. doi: 10.1097/INF.0b013e3181951af4. PMID: 19319014
Factors associated with sclerema in infants with diarrhoeal disease: a matched case-control study.
Chisti MJ, Ahmed T, Faruque AS, Saha S, Salam MA, Islam S
Acta Paediatr 2009 May;98(5):873-8. Epub 2009 Jan 9 doi: 10.1111/j.1651-2227.2008.01196.x. PMID: 19141140
Panniculitis in children.
Torrelo A, Hernández A
Dermatol Clin 2008 Oct;26(4):491-500, vii. doi: 10.1016/j.det.2008.05.010. PMID: 18793982
Normal subcutaneous fat, necrosis of adipocytes and classification of the panniculitides.
Requena L
Semin Cutan Med Surg 2007 Jun;26(2):66-70. doi: 10.1016/j.sder.2007.02.001. PMID: 17544956

Diagnosis

Neonatal Skin Emergencies.
Siegel M, Lee LW
Pediatr Ann 2019 Jan 1;48(1):e36-e42. doi: 10.3928/19382359-20181210-03. PMID: 30653641
Panniculitis: A summary.
Wick MR
Semin Diagn Pathol 2017 May;34(3):261-272. Epub 2016 Dec 27 doi: 10.1053/j.semdp.2016.12.004. PMID: 28129926
Panniculitis in children.
Grassi S, Borroni RG, Brazzelli V
G Ital Dermatol Venereol 2013 Aug;148(4):371-85. PMID: 23900159
Panniculitis in children.
Torrelo A, Hernández A
Dermatol Clin 2008 Oct;26(4):491-500, vii. doi: 10.1016/j.det.2008.05.010. PMID: 18793982
Cold panniculitis.
Quesada-Cortés A, Campos-Muñoz L, Díaz-Díaz RM, Casado-Jiménez M
Dermatol Clin 2008 Oct;26(4):485-9, vii. doi: 10.1016/j.det.2008.05.015. PMID: 18793981

Therapy

Recurrent sclerema in a young infant presenting with severe sepsis and severe pneumonia: an uncommon but extremely life-threatening condition.
Afroze F, Pietroni MA, Chisti MJ
J Health Popul Nutr 2013 Dec;31(4):538-42. doi: 10.3329/jhpn.v31i4.20053. PMID: 24592596Free PMC Article
Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments.
Buster KJ, Burford HN, Stewart FA, Sellheyer K, Hughey LC
Cutis 2013 Aug;92(2):83-7. PMID: 24087781
Panniculitis in children.
Grassi S, Borroni RG, Brazzelli V
G Ital Dermatol Venereol 2013 Aug;148(4):371-85. PMID: 23900159
Risk factors for sclerema neonatorum in preterm neonates in Bangladesh.
Zeb A, Rosenberg RE, Ahmed NU, Saha SK, Chowdhury A, Ahmed S, Darmstadt GL
Pediatr Infect Dis J 2009 May;28(5):435-8. doi: 10.1097/INF.0b013e3181951af4. PMID: 19319014
Cold panniculitis.
Quesada-Cortés A, Campos-Muñoz L, Díaz-Díaz RM, Casado-Jiménez M
Dermatol Clin 2008 Oct;26(4):485-9, vii. doi: 10.1016/j.det.2008.05.015. PMID: 18793981

Prognosis

Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments.
Buster KJ, Burford HN, Stewart FA, Sellheyer K, Hughey LC
Cutis 2013 Aug;92(2):83-7. PMID: 24087781
Panniculitis in children.
Grassi S, Borroni RG, Brazzelli V
G Ital Dermatol Venereol 2013 Aug;148(4):371-85. PMID: 23900159
Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka.
Chisti MJ, Saha S, Roy CN, Ahmed T, Faruque AS, Salam MA, Islam S
Ann Trop Paediatr 2009 Mar;29(1):45-50. doi: 10.1179/146532809X402024. PMID: 19222934
Sclerema neonatorum and subcutaneous fat necrosis of the newborn in the same infant.
Jardine D, Atherton DJ, Trompeter RS
Eur J Pediatr 1990 Dec;150(2):125-6. doi: 10.1007/BF02072054. PMID: 2279508
Sclerema neonatorum and subcutaneous fat necrosis of the newborn.
Fretzin DF, Arias AM
Pediatr Dermatol 1987 Aug;4(2):112-22. doi: 10.1111/j.1525-1470.1987.tb00764.x. PMID: 3309905

Clinical prediction guides

Gemcitabine-associated livedoid thrombotic microangiopathy with associated sclerema neonatorum-like microscopic changes.
Mir-Bonafé JM, Román-Curto C, Santos-Briz A, Cañueto J, Fernández-López E, Unamuno P
J Cutan Pathol 2012 Jul;39(7):707-11. Epub 2012 May 22 doi: 10.1111/j.1600-0560.2012.01918.x. PMID: 22612305
Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka.
Chisti MJ, Saha S, Roy CN, Ahmed T, Faruque AS, Salam MA, Islam S
Ann Trop Paediatr 2009 Mar;29(1):45-50. doi: 10.1179/146532809X402024. PMID: 19222934
Restrictive dermopathy. Report of two affected siblings and a review of the literature.
Welsh KM, Smoller BR, Holbrook KA, Johnston K
Arch Dermatol 1992 Feb;128(2):228-31. doi: 10.1001/archderm.128.2.228. PMID: 1739302
Sclerema neonatorum and subcutaneous fat necrosis of the newborn in the same infant.
Jardine D, Atherton DJ, Trompeter RS
Eur J Pediatr 1990 Dec;150(2):125-6. doi: 10.1007/BF02072054. PMID: 2279508
Sclerema neonatorum. Light and electron microscopic studies.
Pasyk K
Virchows Arch A Pathol Anat Histol 1980;388(1):87-103. doi: 10.1007/BF00430679. PMID: 7467124

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