Neonatal Lupus Erythematosus

Neonatal Lupus Erythematosus

Article
Digestive Health
Heart & Vascular Health
+5
Contributed byLester Fahrner, MD+1 moreJul 26, 2023

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

  • Congenital Lupus
  • Neonatal Cutaneous Lupus Erythematosus
  • Neonatal Lupus Syndrome

What is Neonatal Lupus Erythematosus? (Definition/Background Information)

  • Neonatal Lupus Erythematosus is a form of Subacute Cutaneous Lupus Erythematosus (SCLE) that form a group of autoimmune disorders affecting the skin primarily. The condition occurs in newborn babies of mothers with a history of SCLE
  • Subacute Cutaneous Lupus Erythematosus is an autoimmune disorder of unknown cases that usually affects young women and is characterized by the presence of scaly papules and plaques on the arms and trunk
  • It has to be noted that research scientists and healthcare professionals generally consider Neonatal Lupus Erythematosus neither to be a “true” lupus, nor the infantile form of systemic lupus erythematosus (SLE)
  • Neonatal Lupus Erythematosus is caused by the passage of anti-Ro/SSA and/or anti-La/SSB antibodies across the placenta, leading to the findings in the newborn. Some findings can be detected prenatally, primarily heart conduction defects because of fetal heart rate
  • The onset of symptoms of Neonatal Lupus Erythematosus may be observed within a few days or within 1-2 months after the birth of the child. The condition is also known as Congenital Lupus Erythematosus
  • Children develop characteristic red skin rashes, usually around the face and on the scalp. Additionally, they are at an increased risk for heart abnormalities, such as congenital heart block and other cardiac abnormalities. Hepatosplenomegaly can be seen, which is usually self-limited and benign
  • Neonatal Lupus Erythematosus may be diagnosed through physical examination, complete medical history, electrocardiogram, other cardiac tests, and specialized blood tests. The treatment may involve topical creams for mild skin symptoms. Heart defects may require intervention
  • The prognosis depends on the severity of the disorder and its response to therapy. In general, the prognosis of Neonatal Lupus Erythematosus is good in a majority of children since spontaneous resolutions are observed in many cases

Who gets Neonatal Lupus Erythematosus? (Age and Sex Distribution)

  • Neonatal Lupus Erythematosus is an uncommon condition that is observed in newborn children. The incidence rate is about 1 in 15,000 live births
  • In a few, the signs and symptoms (involving the skin) may not be present at birth but may appear a few days (2-5 days) following the birth of the child
  • Both male and female babies are affected, though females are affected slightly more than males
  • Generally, darker-skinned individuals (Africans and Asians) have a higher risk for cutaneous lupus erythematosus than fair-skinned individuals (Caucasians)

What are the Risk Factors for Neonatal Lupus Erythematosus? (Predisposing Factors)

Risk factors associated with Neonatal Lupus Erythematosus may include:

  • Pregnant women with a history of subacute cutaneous lupus erythematosus (SCLE); however, only about 1 in 50 children born to mothers with SCLE have Congenital Lupus
  • Some mothers can have antibodies to Ro/SSA and/or La/SSB without having signs or symptoms of Subacute Cutaneous Lupus

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 Neonatal Lupus Erythematosus? (Etiology)

The exact cause of Neonatal Lupus Erythematosus, is not fully known or understood.

  • It has been researched that expectant mothers with subacute cutaneous lupus erythematosus, which is an autoimmune disorder, pass on the autoantibodies to the fetus while it is still in the womb
  • An autoimmune disorder is one in which the antibodies of the body that fight diseases or infections attack one’s healthy tissues instead. Such an attack by the body’s immune cells against its tissue can be severe, resulting in significant signs and symptoms

Studies inform that the child with Neonatal Lupus Erythematosus and, in many cases, the mother do not have systemic lupus erythematosus, a severe disorder that can potentially involve several organs and systems of the body.

What are the Signs and Symptoms of Neonatal Lupus Erythematosus?

The signs and symptoms of Neonatal Lupus Erythematosus may vary from one child to another. It may be mild in some and severe in others. In many babies, the symptoms do not appear at birth, but after a few days to some weeks. In a few cases, it may even take 2-5 months for the symptoms to appear.

The signs and symptoms of Neonatal Lupus Erythematosus may include:

  • Presence of a red circular skin lesion (annular rash), most commonly on the eye (periorbital rash). Telangiectasia is sometimes seen
  • Most skin lesions are seen on the face and scalp; some form on the arms, legs, chest, and back
  • These lesions typically form the initial presentations of the syndrome
  • The skin rashes clear spontaneously after a few weeks/months without scarring; they are usually temporary. However, occasionally the rashes may be present into childhood
  • Exposure to sunlight can make the condition worse
  • Many children have defects of the heart that commonly includes congenital heart block with slow heart rate. Heart complications can be first, second, or third degree conduction block
  • Structural heart defects, less common, are patent ductus arteriosus, patent foramen ovale, pulmonic stenosis, and atrial septal defects
  • Children may also develop cardiomyopathy, which is an abnormal condition of the heart muscles, or myocarditis (inflamed myocardium)
  • In some babies, there may be elevated liver function tests due to inflammation of the liver. These changes are generally transient
  • Anemia, neutropenia, thrombocytopenia, and aplastic anemia have been reported
  • Swollen liver and spleen (hepatomegaly and splenomegaly, respectively), with elevated bilirubin, liver enzymes, and cholestasis may be present; these may cause yellowing of skin and eye whites (jaundice)
  • Some brain abnormalities have been reported with an uncertain link to neonatal lupus. These have mostly been found incidentally, without the need for treatment or surgery

It is observed that in some children, both the skin symptoms and heart abnormalities are present; while, in other cases, either the skin symptoms or only the heart abnormalities are observed.

How is Neonatal Lupus Erythematosus Diagnosed?

Neonatal Lupus Erythematosus may be diagnosed using the following tests and exams:

  • Complete physical examination with a comprehensive evaluation of one’s medical history
  • Assessment of the signs and symptoms
  • Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Blood tests that include:
    • Complete blood count (CBC) test to check for anemia
    • Routine blood testing (sedimentation rate/C-reactive protein)
    • Antinuclear antibody (ANA) levels blood test to look for antibodies in blood
    • Serological blood test for anti-double-stranded DNA (ds-DNA)
    • Extractable nuclear antigen antibodies panel blood test to detect anti-Ro antibodies and anti-La antibodies
    • Blood chemistry panels
  • Tests to determine heart function, which may include:
    • Electrocardiogram or EKG that is used to measure the electrical activity of the heart. This gives information regarding the heart rate, strength of contraction, and condition of the walls of the heart
    • Chest X-ray: It is taken to visualize the inside of the chest, including the heart, lung, and blood vessels. This imaging tool can show whether the heart is enlarged or if there is fluid buildup in the lungs
    • Cardiac MRI (magnetic resonance imaging): A technique that uses radio waves, magnetic fields, and a computer screen to create pictures of the heart when it is beating
    • Echocardiography: This procedure uses sound waves to create a motion picture of the heart movement. It can help show the size and shape of the heart and how well the chambers and valves are working
    • Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
  • Tests to determine liver function through the following:
    • Liver function test may be conducted to test for the amount of liver enzymes circulating in blood. When the liver is damaged, an elevated amount of liver enzymes may be detected in blood
    • Imaging tests, such as ultrasound or CT scans, may be performed to examine the liver structure and help detect the presence of any scarring
    • A liver biopsy, if required, may be performed to allow microscopic examination of the liver tissue
  • Prenatal ultrasound scan of the developing fetus
  • Skin/tissue biopsy: A tissue 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 microscope findings, the pathologist arrives at a definitive diagnosis
  • Direct immunofluorescence studies on tissue biopsies

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 Neonatal Lupus Erythematosus?

The complications of Neonatal Lupus Erythematosus may include:

  • Cosmetic concerns causing emotional and psychological stress
  • 1 in 10 children have disease of the liver and bile system (hepatobiliary disease), which may result in liver dysfunction and liver failure
  • 1 in 10 children also have reduced blood counts that lead to anemia, thrombocytopenia (low platelets in the blood), and neutropenia (reduced neutrophils)
  • Rarely, macrocephaly (large head) and hydrocephalus (accumulation of excess fluid in the head) have been observed in newborn children
  • Severe heart abnormalities, such as heart failure and complete heart block, that are even known to be fatal
  • Some affected children are known to develop other autoimmune disorders later in life

How is Neonatal Lupus Erythematosus Treated?

The treatment undertaken for Neonatal Lupus Erythematosus may include:

  • Mild topical medication and ointments, if necessary
  • Systemic corticosteroid therapy for severe cases
  • In those who limit sun exposure, vitamin D supplements may be administered
  • Regular heart evaluation and monitoring; correction of heart blocks and heart defects. In some children, a pacemaker may be necessary
  • Rarely, intravenous immunoglobulin (IVIG) therapy for treating cardiomyopathy
  • Appropriate treatment for hepatobiliary disease
  • Symptomatic and supportive therapy

How can Neonatal Lupus Erythematosus be Prevented?

Instituting adequate treatment measures for underlying subacute cutaneous lupus erythematosus (SCLE) in expectant mothers may help prevent Neonatal Lupus Erythematosus.

  • In pregnant women with SCLE, careful and periodic monitoring of the pregnancy is advised and recommended. It is reported that about 50% of pregnant women with SCLE may not present any apparent symptoms
  • It is also important that close monitoring of both the mother and developing fetus be undertaken
  • Flare-ups can be prevented by managing medications, avoiding sun exposure, utilizing suitable sunscreens and clothing (cover-up skin exposed to sunlight), and close monitoring of the symptoms
  • Educating the individual and keeping them well-informed about lupus is important and beneficial

What is the Prognosis of Neonatal Lupus Erythematosus? (Outcomes/Resolutions)

The prognosis of Neonatal Lupus Erythematosus depends on the severity of the signs and symptoms, its association with heart and liver abnormalities, and the response to treatment.

  • Early intervention and adequate treatment are necessary in children with Neonatal Lupus Erythematosus. It has been noted that not all children with the condition show skin, blood, heart, and liver involvement (full spectrum of the disease)
  • In a majority of affected children, the skin and other associated symptoms resolve in a few months to about 6 months. However, it is important to hold periodic checkups and evaluations to monitor for progression of the disorder
  • Studies inform that about 1 in 5 babies with heart block may die. Therefore, it is important to diagnose heart blocks early (even in the prenatal stage, if possible). Pediatric cardiology consultation is very helpful in babies who are born to mothers with subacute cutaneous lupus erythematosus

Additional and Relevant Useful Information for Neonatal Lupus Erythematosus:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/systemic-lupus-erythematosus/

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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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