ABSTRACT
Neonatal lupus erythematosus (NLE) is caused by the transmission of maternal anti-Ro/SSA antibodies, anti-La/SSB antibodies, and other autoantibodies to the fetus through the placenta. Usually, with the disappearance of autoantibodies in the children's body, abnormal changes in the mucocutaneous, blood system, and digestive system can spontaneously subside, but the damage to various systems caused by autoantibodies may persist for a long time. This article provides a comprehensive review of the manifestations and prognosis of NLE in various systems, including mucocutaneous, blood system, circulatory system, nervous system, digestive system, respiratory system, aiming to provide reference for clinical work.
Subject(s)
Child , Infant, Newborn , Female , Pregnancy , Humans , Lupus Erythematosus, Systemic/diagnosis , Prognosis , Autoantibodies , FamilyABSTRACT
El Lupus Eritematoso Neonatal es una enfermedad de origen autoinmune caracterizada por rash cutáneo transitorio, bloqueo cardíaco congénito permanente, función hepática anormal con o sin enfermedad biliar y compromiso hematológico asociado a la presencia de autoanticuerpos maternos contra la ribonucleoproteinas solubles (SSB/La, SSA/Ro y Anti-RNP). Se presenta el caso de una niña de cinco meses de edad con hallazgos clínicos e histopatológicos de Lupus eritematoso neonatal. Es una condición que no suele dejar secuelas, aunque se han reportado casos de atrofia cutánea e hiperpigmentación
Neonatal Lupus Erythematosus is an autoimmune disease characterized by transitory cutaneous rash, congenital heart block, abnormal liver function test with or without cholestasis and hematologic features associated to anti-Ro and anti-La autoantibodies. A 5-month-old female is brought to the hospital with clinical and histopathology findings of Neonatal lupus. This medical condition does not leave long term physical damage, however there have been cases reported with cutaneous atrophy and hyperpigmentation
Subject(s)
Humans , Female , Infant , Lupus Erythematosus, Cutaneous , Infant, Newborn, Diseases , Autoimmune DiseasesABSTRACT
Neonatal lupus erythematosus (NLE) is an uncommon autoimmune disease that is associated with transplacental passage of maternal autoantibodies into the fetal circulation. It is commonly presented as multiple round, pink to red macules involving scalp, face and extremities. The characteristic skin lesions of NLE generally resolve without scarring within 6 months, but transient hyperpigmentation or hypopigmentation has been reported in some dark-skinned infants. We report a rare case of 3 year-old girl showing telangiectasia at the site of previous NLE lesions. In past history, at 2 days after birth, erythematous macules and patches on face and extremities had been presented and she was diagnosed as NLE in pediatrics. To our knowledge, it is the first report of residual telangiectasia after NLE in Korean dermatologic literature. Furthermore, we propose that dermatologists should consider the possibility of the cutaneous sequelae associated with NLE when encountering a child showing multiple telangiectasia.
Subject(s)
Child , Female , Humans , Infant , Autoantibodies , Autoimmune Diseases , Cicatrix , Extremities , Hyperpigmentation , Hypopigmentation , Parturition , Pediatrics , Scalp , Skin , TelangiectasisABSTRACT
Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Antibodies , Antibodies, Antinuclear , Autoantibodies , Autoimmune Diseases , Biopsy , Dermis , Edema , Epidermis , Heart Block , Liver , Lupus Erythematosus, Cutaneous , Mothers , Serologic Tests , Skin , TelangiectasisABSTRACT
Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Antibodies , Antibodies, Antinuclear , Autoantibodies , Autoimmune Diseases , Biopsy , Dermis , Edema , Epidermis , Heart Block , Liver , Lupus Erythematosus, Cutaneous , Mothers , Serologic Tests , Skin , TelangiectasisABSTRACT
This review summarizes the epidemiology, etiology, clinical manifestation, treatment, follow-up of neonatal lupus erythematosus with focus on new discoveries on the etiology of the disease in recent years including anti-SSA/Ro and anti-SSB/La antibodies, serotonin (5-hydroxytryptamine 4), apoptosis of cardiac cells, calcium channels, maternal micro-chimera, genetic variants, to improve clinician awareness of the disease.
ABSTRACT
The congenital complete atrioventricular block(CCAVB) is a rare disease, which is the most serious complication of neonatal lupus erythematosus. Newborn with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. Transplacental steroid and beta-adrenergic agonist therapies are useful for the first and second degree atrioventricular block. But those therapies are usually not effective for complete atrioventricular block. If the fetus has a complete atrioventricular block, delivery should be considered unless other delivery contraindications. In this situation, early pacemaker implantation surgery can improve the survival rate of patients. We report one case of premature newborn with congenital complete atrioventricular block who is successfully recovered by pacemaker implantation.
Subject(s)
Humans , Infant, Newborn , Adrenergic beta-Agonists , Atrioventricular Block , Cardiac Output , Fetus , Heart Failure , Lupus Erythematosus, Systemic , Rare Diseases , Survival RateABSTRACT
Neonatal lupus erythematosus (NLE) is an autoimmune disease associated with the maternal anti-SSA/Ro and anti-SSB/La antibodies. NLE is characterized by cutaneous erythema, congenital heart block (CHB), hepatic dysfunction and hematological abnormalities. The characteristic cutaneous lesion is an annular or polycyclic erythematous patch or plaque. Individual annular lesions are often about 1 cm in diameter. We report a case of NLE in a 7-day-old female infant, born of a clinically asymptomatic mother, presenting "eye mask" shape rash and telangiectasia on her face. Periorbital rash with "eye mask" appearance presents in a few of NLE cases, and this cutaneous sign has been emphasized as a diagnostic clue to NLE. Her serological tests revealed high titers of anti-SSA/Ro, anti-SSB/La and antinuclear antibody. Based on these clinical and serological findings, the patient was diagnosed as NLE with characteristic periorbital skin findings.
Subject(s)
Female , Humans , Infant , Antibodies , Antibodies, Antinuclear , Autoimmune Diseases , Erythema , Exanthema , Heart Block , Lupus Erythematosus, Systemic , Mothers , Serologic Tests , Skin , TelangiectasisABSTRACT
Neonatal lupus erythematosus (NLE) is an acquired autoimmune disorder caused by the transplacental passage of maternal autoantibodies, usually anti-Ro/SSA or anti-La/SSB antibodies, and less commonly U1-ribonucleoprotein. NLE usually involves a single organ, but multiple organ involvement has also been reported. Manifestations of NLE may include cutaneous lesions, congenital heart block, hematological diseases (anemia, thrombocytopenia, neutropenia), hepatic diseases (hepatitis, hepatosplenomegaly, cholestasis), and neurological diseases. Neurological involvement is very rare in infants with NLE. Here, we report a 2-day-old female neonate, born to a clinically asymptomatic mother, presenting with cutaneous lupus lesions and brain infarction as a neurological disease.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Antibodies , Autoantibodies , Brain , Brain Infarction , Heart Block , Hematologic Diseases , Lupus Erythematosus, Systemic , Mothers , ThrombocytopeniaABSTRACT
Neonatal lupus erythematosus (NLE) is a rare disease characterized by typical clinical features and the transplacental passage of maternal autoantibodies, particularly anti-SSA/Ro. The major clinical manifestations are cutaneous lupus lesions, congenital heart block, hematological disorders, and hepatobiliary diseases. We report a case of NLE presenting with multiple round and oval target-like erythematous skin lesions and abnormal liver function, born to a clinically asymptomatic mother whose diagnosis was made retrospectively only after her newborn's diagnosis. Both the infant and the mother were positive for the anti-SSA/Ro and anti-SSB/La antibodies.
Subject(s)
Humans , Infant , Antibodies , Autoantibodies , Heart Block , Liver , Lupus Erythematosus, Systemic , Mothers , Rare Diseases , Retrospective Studies , SkinABSTRACT
A newborn presented with erythematous lesion over face, which appeared soon after birth. Diagnosis of neonatal lupus erythematosus (NLE) was confirmed by positive anti-Ro SSA antibody and skin biopsy. But anti-La SSB antibody was negative. Her hepatic transaminases were high. But no cardiac manifestations were noted.
Subject(s)
Antibodies, Antinuclear/immunology , Diagnosis, Differential , Face , Female , Humans , Infant, Newborn , Lupus Erythematosus, Cutaneous/diagnosis , Lupus Erythematosus, Cutaneous/immunology , Prognosis , Ribonucleoproteins/immunologyABSTRACT
Neonatal lupus erythematosus (NLE) is an autoimmune disease that is associated with transplacental passage of maternal autoantibodies that are reactive to SSA/Ro and SSB/La antigens. Cardiac involvement, hematologic abnormality and hepatic disease may occur in the infants suffering with NLE, in addition to the characteristic skin lesions. We report here on a case of NLE in a 4-week-old female infant who was born to an asymptomatic mother, and the baby displayed the characteristic clinical and histological features of cutaneous NLE with transient anemia and hepatitis. Both the infant and mother were positive for anti-SSA/Ro and anti-SSB/La. There have been 18 case reports of NLE in the Korean literature, including 7 case reports in the dermatological field. We describe herein another case of NLE that showed transient anemia and hepatitis, and we also review the case reports of NLE in the Korean literature.
Subject(s)
Female , Humans , Infant , Anemia , Autoantibodies , Autoimmune Diseases , Hepatitis , Lupus Erythematosus, Systemic , Mothers , Skin , Stress, PsychologicalABSTRACT
PURPOSE: Neonatal lupus erythematosus (NLE) is a rare disorder caused by the transplacentally transmitted maternal autoantibodies. It is characterized by congenital complete atrioventricular block (CCAVB), skin lesion and less commonly hepatic and hematologic abnormalities. This study was designed to investigate the clinical characteristics and outcome of systemic lupus erythematosus (SLE) mothers and their neonates. METHODS: 29 neonates born to 25 SLE mothers were admitted to the Neonatal Intensive Care Unit of Chonnam University Hospital between January 1994 and June 2005. We investigate the medical records retrospectively. RESULTS: Among 25 SLE mothers, 32.4% had history of spontaneous abortion or stillbirth in previous pregnancy, and 7 (28.0%) of preeclampsia and 3 (12.0%) of premature rupture of membranes in present pregnancy. Among 29 newborns, 14 (48.2%) were premature, 4 (13.7%) were intrauterine growth retardation, and 11 (37.9%) were confirmed as NLE. Anti-SSA/Ro was positive in 13 (52.0%) of 25 examined neonates including 6 (85.7%) of 7 neonates with electrocardiographic abnormalities. Two cases of CCAVB were diagnosed during the pregnancy. One patient didn't show heart failure, he is on the followed up without medical treatment until 4 years of age. The other one showed heart failure, cardiac pacemaker was inserted at 7 days of age, dilated cardiomyopathy developed at 4 years of age. Five neonates with sinus bradycardia resolved spontaneously. One (3.4%) had typical lupus skin lesion with thrombocytopenia and elevated liver enzyme, one (3.4%) had leukopenia, and two (6.9%) had elevated liver enzymes only. CONCLUSIONS: With careful antenatal monitoring of SLE mothers and their fetuses, neonates should be monitored by autoantibody, EKG, and laboratory test.
Subject(s)
Female , Humans , Infant, Newborn , Pregnancy , Abortion, Spontaneous , Atrioventricular Block , Autoantibodies , Bradycardia , Cardiomyopathy, Dilated , Electrocardiography , Fetal Growth Retardation , Fetus , Heart Failure , Intensive Care, Neonatal , Leukopenia , Liver , Lupus Erythematosus, Systemic , Medical Records , Membranes , Mothers , Pre-Eclampsia , Retrospective Studies , Rupture , Skin , Stillbirth , ThrombocytopeniaABSTRACT
Neonatal lupus erythematosus is a rare disorder caused by the transplacental passage of maternal autoantibodies, which result in congenital heart block or cutaneous eruptions similar to lesions of subacute cutaneous lupus erythematosus. We report a case of a 29 day-old-male infant who presented with multiple, targetoid lesions with an edematous margin on the scalp, face, neck, and extremities. His serological tests revealed high titers of anti-La/SSB and antinuclear antibodies. His healthy mother also showed the same high titer of antibodies. Yet, the anti-Ro/SSA antibodies were negative in both of them.
Subject(s)
Humans , Infant , Antibodies , Antibodies, Antinuclear , Autoantibodies , Extremities , Heart Block , Lupus Erythematosus, Cutaneous , Mothers , Neck , Scalp , Serologic TestsABSTRACT
Neonatal lupus erythematosus(NLE) is a distinct subset of lupus characterized by cutaneous findings, cardiac conduction defects, hepatic or hematologic abnormalities. These manifestations are associated with the presence of maternal auto-antibodies such as anti-SSA/Ro, anti-SSB/La, and rarely anti- RNP(U1RNP) antibodies. Cases of U1RNP antibody-positive NLE have somewhat atypical cutaneous manifestation without cardiac or systemic abnormalities. We report a case of cutaneous NLE associated with U1RNP antibodies.
Subject(s)
AntibodiesABSTRACT
Neonatal lupus erythematosus (NLE) is an uncommon passive autoimmune disease in which there is a transplacental passage of anti-Ro or anti-La maternal autoantibodies. The cutaneous lupus lesions are commonly observed and usually resolve spontaneously. The cardiac involvement which sometimes needs the permanent pacemaker, however, is the most clinically significant manifestation of NLE. In addition, the hepatic and hematologic abnormalities are observed in approximately 10% of infants with NLE. Therefore, in the way of the evaluation of hematologic disorders in neonate, NLE should be included. We present a case of NLE in an infant born to a mother with anti-Ro, and he had skin rash, pancytopenia and mildly abnormal liver functions without cardiac manifestation.
Subject(s)
Humans , Infant , Infant, Newborn , Autoantibodies , Autoimmune Diseases , Exanthema , Liver , Mothers , PancytopeniaABSTRACT
Neonatal Lupus Erythematosus (NLE) is a disease of the newborn defined by presence of maternal autoantibodies and characteristic clinical features (congenital complete atrioventricular block, skin lesion, etc.). Although the autoantibodies often are not associated with clinical disease in the mother, NLE is likely the result of fetal or neonatal tissue damage caused by maternally transmitted IgG autoantibodies. The majority of morbidity and mortality of NLE results from congenital complete atrioventricular block. Most of the neonates with NLE have maternally transmitted anti-SSA/Ro antibodies. Anti-SSB/La antibodies are found in 40 to 50 percent of patients. Anti-SSA/Ro antibodies are the most important autoantibodies in the pathophysiology of NLE. Noncardiac manifestations of neonatal lupus are transient, resolving at about 6 months of life, coinciding with the disappearance of maternal autoantibodies from neonatal circulation. Congenital complete atrioventricular block is irreversible, and permanent pacemaker insertion is needed. We report a case of NLE with congenital complete atrioventricular block with literature reviews.
Subject(s)
Humans , Infant, Newborn , Antibodies , Atrioventricular Block , Autoantibodies , Immunoglobulin G , Mortality , Mothers , SkinABSTRACT
Neonatal lupus erytematosus is characterized by cutaneous findings showing the morphology of subacute cutaneous lupus erythematosus, congenital heart block, and anti-Ro/SSA and/or anti-La/SSB autoantibodies which result from the transplacental passage of maternal autoantibodies. We report two cases of a 25-day-old female infant and a 30-day-old male infant who presented with characteristic clinical features of cutaneous neonatal lupus erythematosus but without evidence of congenital heart block. The serological studies in both infants revealed the presence of anti-SSA/Ro antibodies and antinuclear antibodies. We diagnosed their mothers as systemic lupus erythematosus with the clinical and laboratory findings.
Subject(s)
Female , Humans , Infant , Male , Antibodies , Antibodies, Antinuclear , Autoantibodies , Heart Block , Lupus Erythematosus, Cutaneous , Lupus Erythematosus, Systemic , MothersABSTRACT
Neonatal lupus erythematosus(NLE) is a rare disease characterized by the transplacental passage from the mother to the fetus of autoantibodies, particularly autoantibodies of Ro family. The patient with NLE exhibits one or more of the following findings: congenital heart block, cutaneous lupus lesions, hepatobiliary disease and hematologic disorders(thrombocytopenia, anemia). We report a case of NLE in a 2-week-old male infant, born of a clinically asymptomatic mother, presenting multiple, round, target-like lesions which have not been reported in the English and Korean literature. Both infant and mother were positive for anti-SSA/Ro and anti-SSB/La.
Subject(s)
Humans , Infant , Male , Autoantibodies , Fetus , Heart Block , Mothers , Rare Diseases , SkinABSTRACT
Neonatal lupus erythematosus is a syndrome characterized by congenital heart block and photo-distributed papulosquamous eruption, in which multi-organ involvement is not common. We report a case of neonatal lupus erythematosus with pancytopenia and transient bradycardia. The neonate was born to a mother with systemic lupus erythematosus. Both of the neonate and the mother were positive for anti Ro/SS-A antibody.