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Abstract Volume: 1 Issue: 4 ISSN:

Neonatal Lupus: About 2 Cases, One of which Presented with Congenital Heart Block

  Aichaoui H *, Kichou N1, Naceur M2, Bouziane-Nedjadi K3.

*Corresponding Author: Dr. Hayat Aichaoui*, INESSM University, Algeria.


 Received Date:  September 14, 2020

 Publication Date: October 01, 2020

Abstract

Neonatal lupus is very rare, it can occur through the placental passage of maternal antibodies. we report 2 observations of this pathology. A male of 4 months' mother with Sharp's syndrome. Flaky maculopapular lesions in the process of healing in the face, scalp, trunk. Presence of anti-SSA / Ro, speckled Antinuclear, anticardiolipin Ac, Anti RNP antibodies. Regenerative normochromic normocytic anemia. A female of 3 months for the atrioventricular block of antenatal discovery. Rounded scaly erythematous maculopapular lesions on the scalp and face, hepato-splenomegaly. Hepatic cytolysis and cholestasis. Positive anti-SSA / Ro antibodies. Marked by regression of hepatic cytolysis and cholestasis. Treatment by corticosteroids. Good evolution. The neonatal lupus is typical and easily diagnosed. It should be sought in any mother with lupus.

Key Words: Lupus Erythematosus, Cutaneous/Genetics. Antibodies, Antinuclear.

Neonatal Lupus: About 2 Cases, One of which Presented with Congenital Heart Block

Introduction
Neonatal lupus is very rare (approximately one in 17,000 to 20,000 births), this pathology was observed by Hogg in 1957. It can occur through the placental passage of maternal anti-SSA / Ro and anti-SSB / La antibodies., when the mother has lupus or Sjogren’s syndrome, as it can occur in the absence of any disease in the mother. It manifests itself at this age by skin damage, hepatic, hematological, and neurological disorders, but also cardiac disorders through life-threatening BAV. It is a passive acquired autoimmune disorder that progresses favorably, regressing around the age of 6 months.

The Objective of our Work

we report 2 observations of this pathology.

1st Observation

 A male patient referred to our service at the age of 4 months with suspected lupus. The mother with Sharp's syndrome (autoimmune mixed connectivities).

Physical Examination

Flaky maculopapular lesions in the process of healing in the face (periorbital region), scalp, trunk (upper back).

Biology

Regenerative normochromic normocytic anemia.

We note the presence of anti-SSA / Ro, speckled Antinuclear, anticardiolipin Ac, Anti RNP antibodies.

Treatment

The infant was put on topical corticosteroids and emollients.

Evolution

Healing of skin lesions

BAV 1st degree

Disappearance of anemia

Negotiation of anti-SSA / Ro Ab, Antinuclear and anticardiolipin Ab

2nd Observation

A female patient was referred to our ward at the age of 3 months for the atrioventricular block of antenatal discovery.

Physical Examination

The infant presented with rounded scaly erythematous maculopapular lesions on the scalp and face (periorbital and periorbital region), hepato-splenomegaly.

ECG

A complete grade II atrioventricular block.

Biology

Hepatic cytolysis and cholestasis.

The presence of anti-SSA / Ro antibodies in the infant and the mother confirms the maternal origin.

We will also note a vaginal prolapse.

Treatment

Treatment with local corticosteroids was started. Therapeutic abstention for complete AVB has been advocated because of its good tolerance.

The Evolution

Marked by regression of hepatic cytolysis and cholestasis.

Conclusion

The neonatal lupus is typical and easily diagnosed. It should be sought in any mother with lupus given the severity of the AVB which requires in 2/3 of cases the placement of a pacemaker. Also, the evolution of recovery during the first months of life after the disappearance of maternal antibodies.

 

References

1.Zahir AMOURA, Laurent ARNAUD, Alexis MATHIAN “Un modèle d’immunisation fœto-maternelle : le lupus néonatal ?”. Bull. Acad. Natle Méd., 2012, 196, no 8, 1625-1637, séance du 20 novembre 2012.

2.Izmirly P.M., Llanos C., Lee L.A., Askanase A., Kim M.Y., Buyon J.P. “Cutaneous manifestations of neonatal lupus and risk of subsequent congenital heart block”. Arthritis Rheum., 2010, 62, 1153-1157.

3.Ambrosi A., Salomonsson S., Eliasson H. et al. “Development of heart block in children of SSA/SSB-autoantibody-positive women is associated with maternal age and displays a seasonof-birth pattern”. Ann Rheum Dis., 2012, 71, 334-340.

 

 

Volume 1 Issue 4 October 2020

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