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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
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
Subcutaneous fat necrosis of the newborn: a review of 11 cases.
Burden AD, Krafchik BR
Pediatr Dermatol 1999 Sep-Oct;16(5):384-7. doi: 10.1046/j.1525-1470.1999.00101.x. PMID: 10571840
Neonatal peritonitis.
Zorludemir U, Koca M, Olcay I, Yücesan S
Turk J Pediatr 1992 Jul-Sep;34(3):157-66. PMID: 1485383
Blood transfusion therapy in the newborn.
Carapella De Luca E, Stegagno M
Haematologia (Budap) 1981;14(2):173-90. PMID: 7024055

Diagnosis

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 childhood.
Polcari IC, Stein SL
Dermatol Ther 2010 Jul-Aug;23(4):356-67. doi: 10.1111/j.1529-8019.2010.01336.x. PMID: 20666823
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
Scleredema in an infant.
Heilbron B, Saxe N
Arch Dermatol 1986 Dec;122(12):1417-9. PMID: 3789776

Therapy

Panniculitis with crystals induced by etanercept subcutaneous injection.
Llamas-Velasco M, Requena L
J Cutan Pathol 2015 Jun;42(6):413-5. Epub 2015 Apr 1 doi: 10.1111/cup.12478. PMID: 25727404
Panniculitis in children.
Grassi S, Borroni RG, Brazzelli V
G Ital Dermatol Venereol 2013 Aug;148(4):371-85. PMID: 23900159
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
Sclerema neonatorum after therapeutic whole-body hypothermia.
Navarini-Meury S, Schneider J, Bührer C
Arch Dis Child Fetal Neonatal Ed 2007 Jul;92(4):F307. doi: 10.1136/adc.2006.103978. PMID: 17585096Free PMC Article
Perinatal thyroid discharge. A histological study of 1225 infant thyroids.
Sagreiya K, Emery JL
Arch Dis Child 1970 Dec;45(244):746-54. doi: 10.1136/adc.45.244.746. PMID: 5531057Free PMC Article

Prognosis

Panniculitis in children.
Grassi S, Borroni RG, Brazzelli V
G Ital Dermatol Venereol 2013 Aug;148(4):371-85. PMID: 23900159
A firm plaque on the back of a newborn.
Zhou W, Wiesenthal A, Carr V, Allison A, Kelly B, Gibson B
Dermatol Online J 2010 Feb 15;16(2):11. PMID: 20178707
Subcutaneous fat necrosis of the newborn: a review of 11 cases.
Burden AD, Krafchik BR
Pediatr Dermatol 1999 Sep-Oct;16(5):384-7. doi: 10.1046/j.1525-1470.1999.00101.x. PMID: 10571840
Scleredema in an infant.
Heilbron B, Saxe N
Arch Dermatol 1986 Dec;122(12):1417-9. PMID: 3789776
Care of the infant with sclerema neonatorum.
Ferguson CK
JOGN Nurs 1983 Nov-Dec;12(6):391-4. doi: 10.1111/j.1552-6909.1983.tb01091.x. PMID: 6558245

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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