Sclerema Neonatorum

Sclerema Neonatorum

Articlescleremaneonatorum
Skin Care
Diseases & Conditions
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Contributed byLester Fahrner, MD+1 moreJun 13, 2022

What are the other Names for this Condition? (Also known as/Synonyms)

  • SN (Sclerema Neonatorum)

What is Sclerema Neonatorum? (Definition/Background Information)

  • Sclerema Neonatorum (SN) is a severe form of panniculitis that manifests in premature infants, usually within the first week of their life. The term ‘panniculitis’ indicates inflammation of the fat (adipose) tissue
  • Apart from widespread thickening/hardening of the skin, Sclerema Neonatorum causes systemic symptoms that include reduced blood pressure, fluids dysregulation, and hypothermia, which is a medical emergency
  • The cause of Sclerema Neonatorum is unknown. It is seen most frequently in infants with preterm birth, underweight for dates, and congenital abnormalities
  • It can result in significant complications that include septicemia and decrease in respiratory function. The waxy firm fat binds down to the underlying muscle and bone, decreasing chest and abdominal motion
  • Presently, there is no specific treatment for the condition. The treatment measures may include blood transfusion, steroid therapy, and maintenance of fluid balance in the body
  • Despite treatment, the prognosis of Sclerema Neonatorum is poor and the condition often results in death of the infant

Who gets Sclerema Neonatorum? (Age and Sex Distribution)

  • Sclerema Neonatorum is a rare condition that manifests in preterm infants typically within 1-7 days of birth
  • It may be seen in both male and female neonates, although some studies show a male predominance
  • No racial or ethnic group predilection is observed

What are the Risk Factors for Sclerema Neonatorum? (Predisposing Factors)

The following risk factors have been associated with Sclerema Neonatorum:

  • Infants with serious underlying illnesses
  • Decreased oxygenation to the developing fetus during pregnancy, or chronic intrauterine hypoxia
  • Neonatal sepsis
  • Brain hemorrhage
  • Severe electrolyte imbalance in the baby
  • Severe malnutrition
  • Low birth weight
  • Congenital abnormalities
  • Low Apgar scores that indicate severe stress during the birth process
  • Any other factor that may result in premature birth of the child

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Sclerema Neonatorum? (Etiology)

  • The exact cause of Sclerema Neonatorum is currently unknown. Research is being conducted to ascertain the underlying cause
  • Some researchers believe that abnormalities in the fatty acids in the fat tissue either due to abnormal body temperature or decreased oxygenation to the tissues may result in the condition
  • The incidence of Sclerema Neonatorum is lower where advanced  neonatal intensive care is available

What are the Signs and Symptoms of Sclerema Neonatorum?

The signs and symptoms of Sclerema Neonatorum are systemic and the entire body is affected.

  • Skin signs and symptoms may include:
    • Presence of subcutaneous thickening
    • The skin is also waxy and cold to touch
    • There is no pain observed
    • The areas involved are commonly the buttocks, chest, back, and legs; any part of the body skin may be affected
    • Usually, the lower extremities are involved before the trunk is affected
    • The skin presentation is diffuse, symmetric, and extensive
    • The palms, soles, nipples, and genital regions are often spared
    • The condition progresses in the affected infant for up to 4 months
  • Systemic signs and symptoms due to hardening of the skin may include:
    • Decreased blood pressure (hypotension); the blood circulation to the affected skin region is decreased
    • Hypothermia
    • Dehydration
    • Intracranial hemorrhage, which can cause an associated set of symptoms, may be observed

How is Sclerema Neonatorum Diagnosed?

Sclerema Neonatorum may be diagnosed using the following tests and exams:

  • Complete physical examination with comprehensive evaluation of medical history; assessment of the presenting signs and symptoms
  • Tests to diagnose underlying condition in the infant
  • Tissue biopsy: A biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Sclerema Neonatorum?

The complications of Sclerema Neonatorum may include:

  • Illnesses affecting the digestive system
  • Severe respiratory (breathing) difficulties
  • Severe lowering of the pulse rate and body temperature
  • Septicemia (blood stream infection)

How is Sclerema Neonatorum Treated?

Generally, it is difficult to treat Sclerema Neonatorum. Treatment of the condition is undertaken by managing the underlying illness and poor health state of the infant.

The treatment measure for Sclerema Neonatorum may include:

  • Controlling the rate of progression of skin thickening or hardening by using steroids
  • Maintaining fluid and electrolyte balance in the body
  • Undertaking treatment of any associated condition
  • Treating any underlying infection including sepsis through aggressive antibiotic therapy
  • Exchange transfusion (ET), which is a form of blood transfusion
  • Symptomatic treatment and supportive therapy

How can Sclerema Neonatorum be Prevented?

  • Current medical research has not established a method of preventing Sclerema Neonatorum
  • A careful and periodic monitoring of the pregnancy is advised and recommended

What is the Prognosis of Sclerema Neonatorum? (Outcomes/Resolutions)

  • The prognosis of Sclerema Neonatorum is generally poor despite treatment
  • In many cases, even with an aggressive therapy, the condition is known to be fatal

Additional and Relevant Useful Information for Sclerema Neonatorum:

There are many different types of panniculitis and it has been difficult to obtain an accurate picture of their true incidence, since they may be associated with so many conditions. The different types may include:

  • Lupus panniculitis
  • Pancreatitis-associated panniculitis
  • Alpha-1 antitrypsin deficiency associated panniculitis
  • Infective panniculitis
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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Reviewed by

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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