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Year : 2019  |  Volume : 20  |  Issue : 3  |  Page : 276-278

Anti-U1-ribonucleoprotein positive and anti-Ro and anti-La negative neonatal lupus erythematosus

Department of Dermatology, SVS Medical College, Mahbubnagar, Telangana, India

Date of Web Publication28-Jun-2019

Correspondence Address:
Prof. Angoori Gnaneshwar Rao
F12, B8, HIG-II APHB, Baghlingampally, Hyderabad, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_142_18

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Neonatal lupus erythematosus (LE) is associated with anti-Ro and/or anti-La antibodies. Herein, we present a 2-month-old female baby born to an LE mother in evolution. The baby was positive for U1-ribonucleoprotein antibodies and negative for anti-Ro and anti-La antibodies and was not having any systemic involvement.

Keywords: Anti-Ro and anti-La antibodies, neonatal lupus erythematosus, U1-ribonucleoprotein

How to cite this article:
Rao AG, Reddy VS, Aparna K, Haqqani R, Jagadevapuram K, Gupta S, Fathima K, Tejal M, Muppirala D. Anti-U1-ribonucleoprotein positive and anti-Ro and anti-La negative neonatal lupus erythematosus. Indian J Paediatr Dermatol 2019;20:276-8

How to cite this URL:
Rao AG, Reddy VS, Aparna K, Haqqani R, Jagadevapuram K, Gupta S, Fathima K, Tejal M, Muppirala D. Anti-U1-ribonucleoprotein positive and anti-Ro and anti-La negative neonatal lupus erythematosus. Indian J Paediatr Dermatol [serial online] 2019 [cited 2020 Jul 4];20:276-8. Available from: http://www.ijpd.in/text.asp?2019/20/3/276/261873

  Introduction Top

Neonatal lupus erythematosus (NLE) is a rare autoimmune disease due to passage of maternal IgG autoantibodies, mostly anti-Ro/SSA and anti-La/SSB and U1-ribonucleoprotein (RNP) antibodies. The incidence of NLE is 1 in 20,000 American live births. It has been reported that 50% of mothers with NLE infants may be asymptomatic.[1] It can present with heart blocks and skin, hepatic, or hematological manifestations.

  Case Report Top

A 2-month-old female baby was brought to the Dermatology Department with a history of erythematous scaly patches around both the eyes of 1-month duration. The baby was born at term by lower-segment cesarean section to apparently normal mother. The infant was normal at birth; a month later, the mother noticed erythematous patches over the inner aspect of both eyes and gradually spread to involve the entire periorbital area. There was no history of any topical application or instillation of eye drops. The mother was not a known case of LE, and she did not give a history of joint pain, Raynaud's phenomenon, photosensitive rash, convulsions, chest pain, muscle pain, and oral ulcers.

General examination revealed a well-nourished baby with no pallor, icterus, cyanosis, clubbing, koilonychia, or lymphadenopathy. Cutaneous examination revealed well-demarcated annular, erythematous scaly patches around both the eyes and single annular erythematous plaque over the left cheek [Figure 1], [Figure 2], [Figure 3]. There was neither atrophy nor telangiectasia. Systemic examination was unremarkable. She was provisionally diagnosed as subacute cutaneous lupus erythematosus (SCLE) and NLE. Hartnup disease was kept in the differential diagnosis. However, Hartnup disease was ruled out as there were no seizures, nystagmus, or hypotonia in the index case. Routine hematological, biochemical investigations, chest skiagram, electrocardiogram, two-dimensional (2D) echocardiography, and ultrasonography of the abdomen were unremarkable. Serological investigations revealed positive antinuclear antibodies (ANA) (2.5) and positive anti-U1RNP antibodies (3+). However, anti-Ro and anti-La antibodies, anti-dsDNA antibodies, anticentromere antibodies, anti-Scl 70 antibodies, and anti-Mi2 antibodies were negative. Venereal disease research laboratory test (VDRL) and human immune deficiency virus (HIV) serology were nonreactive. Skin biopsy and direct immunofluorescence (DIF) test could not be done as the mother did not give consent. Examination of the mother revealed no clinical evidence of any connective tissue disease. Serology of the mother showed positive ANA (3.92), anti-dsDNA (2.96), and anti-U1RNP antibodies (3+). Anti-Ro and anti-La antibodies, anticentromere antibodies, anti-Scl 70 antibodies, and anti-Mi2 antibodies were negative. VDRL test and HIV serology were nonreactive. She did not give consent for skin biopsy and DIF study. She was diagnosed as LE in evolution based on serological findings. She was further investigated for evidence of systemic involvement which included hematological and biochemical investigations, urine analysis, chest skiagram, electrocardiogram, 2D echocardiography, and ultrasonography of the abdomen. However, the results revealed normal study. In view of the positive U1RNP antibodies in both mother and infant, possibility of both mixed connective tissue disease (MCTD) and LE in evolution in mother and NLE and MCTD in infant were considered. As the mother is LE in evolution and typical periorbital annular scaly rash in the baby, the diagnosis of NLE was considered. The baby was finally diagnosed as anti U1RNP positive and anti-Ro and anti-La negative NLE, and the mother was diagnosed as LE in evolution. As there was no evidence of systemic involvement in both baby and mother, they were advised photoprotection and were kept under observation and follow-up.
Figure 1: Erythematous scaly patches around both eyes

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Figure 2: Annular erythematous scaly patch on the left cheek

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Figure 3: Close-up view of erythematous scaly patches around both the eyes

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  Discussion Top

McCuistion et al. first described typical lupus eruption in a newborn infant in 1984.[1]

The presence of human leukocyte antigen B8 (HLA-B8) and HLA-DR3 in mother predisposes the infant to NLE and congenital heart block. NLE is known to occur in both boys and girls equally.[2] Most cases have either cardiac lesions or skin lesions, and approximately 10% have both.[3] The spectrum of clinical features of NLE includes transient rash, congenital heart block (CHB), hepatomegaly, pneumonitis, lymphadenopathy, thrombocytopenia, and hemolytic anemia. The rash usually appears in the first 2 months of birth. Similarly, the reported case also presented with rash in neonatal period. Annular erythematous plaques with slight scaling are characteristic of NLE.[4] Characteristically, the rash develops on sun-exposed areas such as face, arms, and scalp. Often, the rash is annular and circumscribed resembling adult lesions of SCLE. The annular scaly patches surrounding the eyes in the reported case are characteristic of NLE. The skin lesions usually disappear in 6–9 months. Heart is affected in 60% of cases, complete heart block (CHB) being the most common (15%–30%) due to fibrosis of bundle of His and is usually permanent and require a pacemaker.[5] Mortality rate with cardiac involvement is 20%, and 10% of NLE patients have hepatic involvement, and 10% have hemolytic anemia, thrombocytopenia, and neutropenia. Favorably, children with NLE are not at increased risk of development of systematic LE.

Majority of infants with NLE are associated with anti-Ro/SSA and anti-La/SSB antibodies. The absence of these antibodies in the index case is notable. Solomon et al.[6] reported three cases of U1RNP-positive NLE with cutaneous lesions similar to the anti-Ro-positive NLE, although lacked systemic abnormalities. Sheth et al.[7] and Frey et al.[8] also reported similar observations in their case studies [Table 1]. The index case is in concert with the case reports documented in the literature as it is also U1RNP positive and anti-Ro/anti-La negative and devoid of systemic manifestations. Moreover, it is known that anti-Ro antibodies influence development of heart block. The absence of heart block in the index case may be attributed to the absence of anti-Ro antibodies. However, the case requires further observation for systemic manifestations such as cardiac conduction defects. The prognosis is good in the index case as there is no systemic involvement. Nonetheless, regular follow-up is required until adolescence. Future pregnancies should be monitored by serial echocardiography. Dexamethasone and intravenous immunoglobulins have been used with success in the management of heart block in NLE in utero.[9]
Table 1: U1RNP positive and Anti Ro/Anti La negative NLE cases reported in literature

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Conflicts of interest

There are no conflicts of interest.

  References Top

Lee LA. Neonatal lupus erythematosus: Clinical findings and pathogenesis. J Investig Dermatol Symp Proc 2004;9:52-6.  Back to cited text no. 1
David GP. Dermatoses of the neonate. In: Christopher EM, Barker J, Bleiter T, Chalmers R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. Oxford: Blackwell Publishing, Ltd.; 2016. p. 116.12-13.  Back to cited text no. 2
Boh EE. Neonatal lupus erythematosus. Clin Dermatol 2004;22:125-8.  Back to cited text no. 3
Neiman AR, Lee LA, Weston WL, Buyon JP. Cutaneous manifestations of neonatal lupus without heart block: Characteristics of mothers and children enrolled in a national registry. J Pediatr 2000;137:674-80.  Back to cited text no. 4
Jenkins RE, Kurwa AR, Atherton DJ, Black MM. Neonatal lupus erythematosus. Clin Exp Dermatol 1994;19:409-11.  Back to cited text no. 5
Solomon BA, Laude TA, Shalita AR. Neonatal lupus erythematosus: Discordant disease expression of U1RNP-positive antibodies in fraternal twins – Is this a subset of neonatal lupus erythematosus or a new distinct syndrome? J Am Acad Dermatol 1995;32:858-62.  Back to cited text no. 6
Sheth AP, Esterly NB, Ratoosh SL, Smith JP, Hebert AA, Silverman E, et al. U1RNP positive neonatal lupus erythematosus: Association with anti-La antibodies? Br J Dermatol 1995;132:520-6.  Back to cited text no. 7
Frey MN, Ioppi AE, Garbin GC, Furian RD, Bau AE. Congenital and neonatal lupus erythematosus: Two case reports. An Bras Dermatol 2012;87:625-8.  Back to cited text no. 8
Trucco SM, Jaeggi E, Cuneo B, Moon-Grady AJ, Silverman E, Silverman N, et al. Use of intravenous gamma globulin and corticosteroids in the treatment of maternal autoantibody-mediated cardiomyopathy. J Am Coll Cardiol 2011;57:715-23.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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