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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neonatal lupus erythematosus (NLE) is an uncommon disease of the neonate. It is believed to be caused by the transplacental passage of maternal autoantibodies to the ribonucleoproteins (Ro/SSA, La/SSB or rarely U RNP) as these are almost invariably present in NLE sera. The most common clinical manifestations include cutaneous lupus lesions and congenital complete heart block. Hepatobiliary and haematologic abnormalities are reported less frequently. We describe a patient with cutaneous NLE to illustrate and raise awareness of the characteristic annular eruption of this condition. We also emphasize the need for thorough investigation for concomitant organ involvement and for maternal education regarding risk in future pregnancies.
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PMID:Rings in the neonate. 2146 78

Neonatal lupus erythematosus (NLE) is an uncommon condition usually associated with maternal anti-Ro autoantibodies. The cutaneous lesions of NLE are usually transient, disappearing about six months after birth, but telangiectasia is a rare complication of NLE which persists. Telangiectasias are small focal red macules and papules created by abnormally prominent capillaries, venules, and arterioles and are a characteristic marker of connective tissue diseases. We report the case of an infant diagnosed with NLE presenting typical annular lesions, positive ANA and positive anti-Ro antibodies. By five months of age, both ANA and anti-Ro antibodies were negative and the annular cutaneous lesions had gradually faded, but small scattered focal red macules appeared on the face, especially in the peri-orbital area and scalp. The cutaneous lupus disappeared but the telangiectasia persisted. We would like to suggest that the diagnosis of NLE should be considered when cutaneous telangiectasias are observed in infants.
Lupus 2012 Apr
PMID:Facial telangiectasia: an unusual manifestation of neonatal lupus erythematosus. 2217 Jul 60

Within the last decade the prevalence of adult patients living with congenital heart disease equals that seen in children. This expanding population poses a challenge to clinical cardiologists who will be caring for patients with the clinical manifestations of this condition. Neonatal lupus is a model of passively acquired autoimmunity and is responsible for the majority of clinical cases of congenital heart block (CHB). This review will focus on the presentation, pathophysiology, and the long-term follow-up of CHB associated with neonatal lupus, and discuss important diagnostic tests, familial implications, and pacemaker issues associated with the care of an adult with CHB.
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PMID:Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population. 2218 63

Neonatal lupus erythematosus (NLE) is an autoimmune disease affecting the fetus as a result of transplacental transfer of anti-Ro autoantibodies. Typically, it presents in the first few months of life with an annular form of subacute cutaneous lupus erythematosus. We report an unusual case of NLE presenting at birth with scaly erythematous telangiectatic patches and macules with skin atrophy involving the face, head, and upper trunk. Thrombocytopenia was discovered on laboratory investigations. Histopathology of skin biopsy was consistent with subacute cutaneous lupus. The mother was clinically free of disease and had no family history of autoimmune disease. Serology (extra-nuclear antigens) was positive in both the baby and the mother. This is a rare presentation of a rare disease.
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PMID:Congenital lupus erythematosus. 2234 83

Neonatal autoimmune diseases are distinctly rare. Most neonatal autoimmune diseases result from the transplacental transfer of maternal antibodies directed against fetal or neonatal antigens in various tissues. In neonatal lupus, the heart seems to be particularly susceptible. Primary autoimmunity in newborns, with the exception of familial autoinflammatory diseases, is virtually non-existent. The pathophysiologic basis for the development of neonatal autoimmunity is not entirely clear, but differences in the neonatal immune system compared with the adult immune system, as well as unique characteristics of target antigens in the newborn period may be important factors. Neonatal lupus is the most common presentation of autoimmunity in the newborn. But the characteristics defining neonatal lupus are not well defined and the presentation of neonatal lupus differs from that of classical lupus. Other neonatal autoimmune diseases involving the interaction between maternal antibodies and fetal/neonatal antigens include neonatal anti-phospholipid syndrome, Behcet's disease, neonatal autoimmune thyroid disease, neonatal polymyositis and dermatomyositis, neonatal scleroderma and neonatal type I diabetes mellitus. While autoantibodies have been detected in patients with neonatal autoimmune disease, the pathogenic role of autoantibodies has not been well defined. Other mechanisms may play a role in the development of neonatal autoimmunity, including fetal/maternal microchimerism and aberrant apoptosis of fetal cells. The autoinflammatory syndromes are a completely different category, but are also included in discussion of neonatal autoimmune diseases. The autoinflammatory syndromes include the cryopyrin associated periodic syndromes (CAPS) - familial cold autoinflammatory syndrome (FCAS), neonatal onset multisystem inflammatory disease (NOMID) and Muckle-Wells syndrome, which all share a common pathophysiologic mechanism.
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PMID:Neonatal autoimmune diseases: a critical review. 2240 39

Neonatal lupus is a rare syndrome resulting from passively transferred maternal autoantibodies during pregnancy. A male infant was delivered at term to a 29-year-old primiparous woman who was diagnosed of systemic lupus erythematosus 2 years earlier and had detectable levels of autoantibodies (antinuclear antibody (ANA), anti-dsDNA, anti-Ro and anti-La/SSB) in second trimester. However, the pregnancy was otherwise uneventful. He presented at the age of 8 week with a widespread hypopigmented macular rash on the trunk and patchy alopecia involving the hair line and the occipito-parietal regions of 3 weeks duration, anaemia and symptomatic thrombocytopaenia. Serologic test for autoantibodies was positive for ANA and anti-La/SSB. Further evaluation was normal. He was managed conservatively with blood products and topical corticosteroids. Mother was also advised to avoid direct exposure to sunlight and fluorescent light. Haematological parameters gradually normalised over 2 months and the skin lesions resolved completely by the age of 6 months.
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PMID:Neonatal lupus syndrome in a Nigerian child. 2260 70

Neonatal lupus erythematosus is an autoimmune disease produced by the passage of maternal antinuclear antibodies and extractable nuclear antigen antibodies through the placenta. At the moment of the diagnosis, the mothers are asymptomatic in 40 to 60% of cases. The most common manifestations are cutaneous lesions and congenital heart block. The cutaneous findings are variable and usually begin within the first weeks or months of life. Congenital lupus erythematosus is a congenital variant of neonatal lupus erythematosus. We present one case of congenital lupus erythematosus and one case of neonatal lupus erythematous, showing the variability of this disease.
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PMID:Congenital and neonatal lupus erythematosus: two case reports. 2289 81

Neonatal lupus is a passively acquired autoimmune syndrome resulting from the transplacental passage of maternal anti-Ro/SSA and/or anti-La/SSB antibodies to the fetus. Few past studies have reported central nervous system involvement in neonatal lupus, and most cases had a good neurological outcome. We report here a preterm case of neonatal lupus with thrombocytopenia and comorbid hemorrhagic stroke. In the follow-up, the infant developed spastic quadriplegia and showed delayed milestones. We believe that this is the first reported case of neonatal lupus accompanied by perinatal hemorrhagic stroke. We present this case to remind clinicians to conduct regular central nervous system surveys in cases of neonatal lupus.
Lupus 2012 Dec
PMID:Neonatal lupus complicated by hemorrhagic stroke. 2316 79

Neonatal lupus erythematosus (NLE) is a rare acquired autoimmune disease caused by transplacental transfer of maternal immunoglobulin G antibodies to the fetus. NLE has well-recognized cutaneous features and may also manifest in other organs. The majority of cases are associated with Ro/SSA and La/SSB antibodies. Neonatal lupus due to antiribonucleoprotein (RNP) antibodies has rarely been reported. On rare occasions RNP has been found in association with other antibodies. We report a case of NLE occurring solely due to RNP antibodies presenting as varicelliform lesions at birth. We recorded the features in our case and 14 additional cases identified in the literature. It is important to recognize that maternal transfer of RNP antibodies may produce the classic cutaneous features of neonatal lupus. The limited case reports of this condition suggest that manifestations are limited to the skin; specifically, there are no reports of cardiac involvement. The long-term outcome remains unknown. RNP-positive, Ro/La-negative NLE seems to represent a different clinical subset of NLE. The recognition of RNP antibody NLE as a benign condition limited to the skin is helpful in planning antenatal care for subsequent pregnancies.
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PMID:Neonatal lupus syndrome associated with ribonucleoprotein antibodies. 2343 84

Neonatal lupus is a rare disease caused by the transplacental transfer of maternal autoantibodies to the foetus, characterized by transient clinical manifestations such as cutaneous, haematological, and hepatobiliary events or permanent such as congenital heart block. The typical cutaneous manifestations include erythematous, scaly, annular or arched lesions on the face, with slight central atrophy and photosensitivy, clinically and histologically similar to subacute cutaneous lupus. However, in some cases, the lesions may resemble those in cutis marmorata telangiectatica congenita, although this phenomenon is rare and only eight such cases have been reported to date. We report a case of cutaneous neonatal lupus with atypical lesions on the limbs, which had a reddish-purple marbled appearance, resembling the lesions in cutis marmorata telangiectatica congenita.
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PMID:Cutaneous neonatal lupus with cutis marmorata telangiectatica congenita-like lesions. 2379 3


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