Neonatal and Pediatric Lupus Erythematosus Differential Diagnoses
- Author: Jeffrey P Callen, MD; Chief Editor: Lawrence K Jung, MD more...
Diagnostic Considerations
At birth, the 4 main considerations in the differential diagnosis of neonatal lupus erythematosus (NLE) are usually congenital rubella, congenital syphilis, Bloom syndrome, and Rothmund-Thomson syndrome. Later, slightly atrophic vascular patches may resemble other disorders, including lymphangiomas.
The following conditions should also be considered when evaluating a patient with suspected neonatal lupus erythematosus:
- Atrioventricular block
- Drug eruptions
- Juvenile dermatomyositis
- Juvenile sarcoidosis
- Malignancy
- Milia
- Primary immunodeficiencies
- Toxic exposures
- Viral or bacterial infections
Differentials
- Dermatologic Manifestations of Juvenile Xanthogranuloma
- Erythema Annulare Centrifugum
- Erythema Infectiosum (Fifth Disease)
- Erythema Toxicum Neonatorum
- Juvenile Idiopathic Arthritis
- Lymphocytoma Cutis
- Pediatric Mixed Connective Tissue Disease
- Pediatric Sjogren Syndrome
- Polyarteritis Nodosa
- Subacute Cutaneous Lupus Erythematosus
Eftekhari P, Sallé L, Lezoualc'h F, Mialet J, Gastineau M, Briand JP, et al. Anti-SSA/Ro52 autoantibodies blocking the cardiac 5-HT4 serotoninergic receptor could explain neonatal lupus congenital heart block. Eur J Immunol. Oct 2000;30(10):2782-90. [Medline].
Boutjdir M, Chen L, Zhang ZH, Tseng CE, El-Sherif N, Buyon JP. Serum and immunoglobulin G from the mother of a child with congenital heart block induce conduction abnormalities and inhibit L-type calcium channels in a rat heart model. Pediatr Res. Jul 1998;44(1):11-9. [Medline].
Yang Q, Shao XM, Cao Y, Chen C, Cheng GQ, Shi YY, et al. [Neonatal lupus erythematosus: analysis of 8 cases]. Zhonghua Er Ke Za Zhi. Jan 2008;46(1):18-21. [Medline].
Rivera TL, Izmirly PM, Birnbaum BK, et al. Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus. Ann Rheum Dis. Jun 2009;68(6):828-35. [Medline].
Spence D, Hornberger L, Hamilton R, Silverman ED. Increased risk of complete congenital heart block in infants born to women with hypothyroidism and anti-Ro and/or anti-La antibodies. J Rheumatol. Jan 2006;33(1):167-70. [Medline].
Peñate Y, Luján D, Rodríguez J, Hernández-Machín B, Montenegro T, Afonso JL, et al. [Neonatal lupus erythematosus: 4 cases and clinical review]. Actas Dermosifiliogr. Dec 2005;96(10):690-6. [Medline].
Asboth D, Kassay E, Noll J, Szalai Z. Neonatal lupus erythematosus: deep and ulcerating form. Borgyogaszati Venerol Szemle. 2000;76:263-5.
Lynn Cheng C, Galbraith S, Holland K. Congenital lupus erythematosus presenting at birth with widespread erosions, pancytopenia, and subsequent hepatobiliary disease. Pediatr Dermatol. Jan-Feb 2010;27(1):109-11. [Medline].
Elish D, Silverberg NB. Neonatal lupus erythematosus. Cutis. Feb 2006;77(2):82-6. [Medline].
Cimaz R, Biggioggero M, Catelli L, Muratori S, Cambiaghi S. Ultraviolet light exposure is not a requirement for the development of cutaneous neonatal lupus. Lupus. 2002;11(4):257-60. [Medline].
Floristan U, Feltes R, González-Beato M, Feito M, Laguna Rde L. Targetoid lesions and neutrophilic dermatosis within neonatal lupus erythematosus: unusual clinical and histologic presentations. J Cutan Med Surg. Jan-Feb 2010;14(1):46-7. [Medline].
Wisuthsarewong W, Soongswang J, Chantorn R. Neonatal lupus erythematosus: clinical character, investigation, and outcome. Pediatr Dermatol. Mar-Apr 2011;28(2):115-21. [Medline].
Brucato A, Frassi M, Franceschini F, Cimaz R, Faden D, Pisoni MP, et al. Risk of congenital complete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis: a prospective study of 100 women. Arthritis Rheum. Aug 2001;44(8):1832-5. [Medline].
Guettrot-Imbert G, Cohen L, Fermont L, Villain E, Francès C, Thiebaugeorges O, et al. A new presentation of neonatal lupus: 5 cases of isolated mild endocardial fibroelastosis associated with maternal Anti-SSA/Ro and Anti-SSB/La antibodies. J Rheumatol. Feb 2011;38(2):378-86. [Medline].
Boros CA, Spence D, Blaser S, Silverman ED. Hydrocephalus and macrocephaly: new manifestations of neonatal lupus erythematosus. Arthritis Rheum. Mar 15 2007;57(2):261-6. [Medline].
Demaestri M, Sciascia S, Kuzenko A, Bergia R, Barberis L, Lanza MG, et al. Neonatal lupus in triplet pregnancy of a patient with undifferentiated connective tissue disease evolving to systemic lupus erythematosus. Lupus. Apr 2009;18(4):368-71. [Medline].
Yang CH, Chen JY, Lee SC, Luo SF. Successful preventive treatment of congenital heart block during pregnancy in a woman with systemic lupus erythematosus and anti-Sjögren's syndrome A/Ro antibody. J Microbiol Immunol Infect. Oct 2005;38(5):365-9. [Medline].
Buyon JP, Clancy RM, Friedman DM. Cardiac manifestations of neonatal lupus erythematosus: guidelines to management, integrating clues from the bench and bedside. Nat Clin Pract Rheumatol. Mar 2009;5(3):139-48. [Medline].
Friedman DM, Llanos C, Izmirly PM, et al. Evaluation of fetuses in a study of intravenous immunoglobulin as preventive therapy for congenital heart block: Results of a multicenter, prospective, open-label clinical trial. Arthritis Rheum. Apr 2010;62(4):1138-46. [Medline].
Pisoni CN, Brucato A, Ruffatti A, et al. Failure of intravenous immunoglobulin to prevent congenital heart block: Findings of a multicenter, prospective, observational study. Arthritis Rheum. Apr 2010;62(4):1147-52. [Medline].
Izmirly PM, Kim MY, Llanos C, et al. Evaluation of the risk of anti-SSA/Ro-SSB/La antibody-associated cardiac manifestations of neonatal lupus in fetuses of mothers with systemic lupus erythematosus exposed to hydroxychloroquine. Ann Rheum Dis. Oct 2010;69(10):1827-30. [Medline].
Shinohara K, Miyagawa S, Fujita T, Aono T, Kidoguchi K. Neonatal lupus erythematosus: results of maternal corticosteroid therapy. Obstet Gynecol. Jun 1999;93(6):952-7. [Medline].
Martin V, Lee LA, Askanase AD, Katholi M, Buyon JP. Long-term followup of children with neonatal lupus and their unaffected siblings. Arthritis Rheum. Sep 2002;46(9):2377-83. [Medline].
Tincani A, Rebaioli CB, Frassi M, Taglietti M, Gorla R, Cavazzana I, et al. Pregnancy and autoimmunity: maternal treatment and maternal disease influence on pregnancy outcome. Autoimmun Rev. Sep 2005;4(7):423-8. [Medline].
Costedoat-Chalumeau N, Amoura Z, Villain E, Cohen L, Piette JC. Anti-SSA/Ro antibodies and the heart: more than complete congenital heart block? A review of electrocardiographic and myocardial abnormalities and of treatment options. Arthritis Res Ther. 2005;7(2):69-73. [Medline]. [Full Text].
Fritsch C, Hoebeke J, Dali H, Ricchiuti V, Isenberg DA, Meyer O, et al. 52-kDa Ro/SSA epitopes preferentially recognized by antibodies from mothers of children with neonatal lupus and congenital heart block. Arthritis Res Ther. 2006;8(1):R4. [Medline]. [Full Text].
Lawrence S, Luy L, Laxer R, Krafchik B, Silverman E. The health of mothers of children with cutaneous neonatal lupus erythematosus differs from that of mothers of children with congenital heart block. Am J Med. Jun 15 2000;108(9):705-9. [Medline].
Lee LA. Transient autoimmunity related to maternal autoantibodies: neonatal lupus. Autoimmun Rev. Apr 2005;4(4):207-13. [Medline].
Yang CH, Chen JY, Lee SC, Luo SF. Successful preventive treatment of congenital heart block during pregnancy in a woman with systemic lupus erythematosus and anti-Sjögren's syndrome A/Ro antibody. J Microbiol Immunol Infect. Oct 2005;38(5):365-9. [Medline].

