Sclerema adiposum neonatorum P83.0

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

History
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Uzembenzius, 1772; Underwood, 1784

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

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

Manifestation
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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.

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

Clinical features
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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.

Histology
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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 diagnosis
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Complication(s)
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Risk of septic processes.

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

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

Literature
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  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

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020