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Sclerema adiposum neonatorumP83.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Adiposclerosis of the newborn; Adiposclerosis of the newborns; Sclerema; Sclerema neonatorum; Sclerodermatous skin changes in an Infant; Scleroedema neonatorum; Underwood's disease

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HistoryThis section has been translated automatically.

Uzembenzius, 1772; Underwood, 1784

DefinitionThis section has been translated automatically.

Rare, diffuse, indurating panniculitis of immature newborns, usually occurring secondarily as a symptom of a life-threatening underlying disease.

EtiopathogenesisThis section has been translated automatically.

Unsolved. Discussed are inadequate nutrition, infections, diarrhoea, cooling (observed after therapeutic hypothermia).

ManifestationThis section has been translated automatically.

Occurs from birth, especially between the 2nd and 10th day of life, in severe underlying diseases also in older children. There is a slight predominance of the male sex.

LocalizationThis section has been translated automatically.

The thighs and buttocks are affected first. Rapid generalization. Palmae and Plantae are free.

Clinical featuresThis section has been translated automatically.

Leather-like hardened, waxy pale, cold, possibly also cyanotic, non-removable skin and subcutaneous tissue. No dents can be pressed in. Mask-like rigid face, impaired mobility of joints, restricted breathing excursion, lowered body temperature.

HistologyThis section has been translated automatically.

Widened connective tissue septums, enlarged, irregular fat cells with "needle shaped clefts", needle-shaped crystals consisting of triglycerides; typically, an inflammatory infiltrate is missing, apart from some neutrophil and eosinophilic granulocytes, macrophages or multinucleated giant cells.

Differential diagnosisThis section has been translated automatically.

Adiponecrosis subcutanea neonatorum.

Complication(s)This section has been translated automatically.

Risk of septic processes.

TherapyThis section has been translated automatically.

Symptomatic intensive medical care with circulation stabilisation, fluid and electrolyte stabilisation, regulation of body heat (incubator), tube feeding.

Progression/forecastThis section has been translated automatically.

Awkward. Rapid lethal outcome is possible. Mortality 50 to 85%. Death by sepsis.

LiteratureThis section has been translated automatically.

  1. Battin M et al (2002) Sclerema Neonatorum following hypothermia. J Paediatr Child Health 38: 533-534
  2. Darragh CT et al (2014) Sclerodermatous skin changes in an infant. Pediatric Dermatol 31:387-388
  3. de Silva U et al (1994) Historical approach to scleroderma. Clin Dermatol 12: 201-205
  4. Llamas-Velasco M et al (2015) Panniculitis with crystals induced by etanercept subcutaneous injection. J Cutan Pathol doi: 10.1111/cup.12478
  5. Navarini-Meury S et al (2007) Sclerema neonatorum after therapeutic whole-body hypothermia. Arch Dis Child Fetal Neonatal Ed 92: F307
  6. Requena L et al (2001) Panniculitis. Part II. Mostly lobular panniculitis. J Am Acad Dermatol 45: 325-361

  7. Shrestha S et al (2017) Sclerema Neonatorum Treated Successfully with Parenteral Steroids: An Experience from a Resource Poor Country.Case Rep Pediatr 2017:4836142.

  8. Warwick W et al (1963) Sclerema neonatorum - a sign, not a disease. JAMA 184: 680-683
  9. Zeb A et al (2008) Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management. J Perinatol 28:453-460

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer