Skip to main content Skip to main navigation menu Skip to site footer

Neonatal lupus erythematosus: sebuah laporan kasus

  • Dewa Ayu Desy ,
  • Ketut Dewi Kumara Wati ,
  • Putu Junara Putra ,

Abstract

Background: The Neonatal Lupus Erythematosus (NLE) phenotype is characterized by a clinical spectrum of skin, heart, and general features observed in the early infancy of infants born to mothers with autoantibodies against Ro/SSA, La/SSB, and U1 ribonucleoprotein (U1-RNP). The diagnosis is supported by clinical symptoms and identifying NLE-associated antibodies in either the mother's or infant's serum. We aim to report clinically manifest cases of NLE to increase knowledge about NLE management and outcome.

Case Presentation: A 4-day-old boy complained of bloody vomiting and bruises on both eyes, abdomen and thighs since birth. The patient was found to have a red rash on the cheeks that crossed the nose line, which was shaped like a butterfly. The patient was taken from systemic lupus erythematosus mothers, who was diagnosed with SLE in 2012 and got therapy until 2014. On the first day of admission, the patient seemed lethargic. The physical examination revealed bruised (periorbital erythema) and subconjunctiva bleeding on both eyes, bruised on abdomen and thigh, and butterfly rash on the cheek, but no bulging fontanelle. The ECG was within normal limits, and the echocardiogram revealed a left-to-right shunt due to a patent foramen ovale (PFO) and mild tricuspid regurgitation, neither requiring cardiac treatment. 1:100 ANA IF titer with a speckled pattern, positive ANA profile histon, anti-DS DNA 60.7 IU/mL, and C3 124.4 mg/dL. The patient was subsequently diagnosed with mild to moderate neonatal lupus erythematosus based on clinical history, physical exam, and laboratory examination.

Conclusion: Neonatal lupus erythematosus (NLE) can be fatal; therefore, observation is crucial.

 

Latar belakang: Fenotipe Neonatal Lupus Erythematosus (NLE) ditandai dengan spektrum klinis kulit, jantung, dan gambaran umum yang diamati pada awal masa bayi bayi yang lahir dari ibu dengan autoantibodi terhadap ribonukleoprotein Ro/SSA, La/SSB, dan U1. (U1-RNP). Diagnosis didukung oleh adanya gejala klinis dan identifikasi antibodi terkait NLE baik dalam serum ibu atau bayi. Penulis bertujuan untuk melaporkan kasus NLE secara klinis guna meningkatkan pengetahuan terkait manajemen dan hasil NLE.

Presentasi Kasus: Seorang anak laki-laki berusia 4 hari datang dengan keluhan muntah darah dan memar yang muncul pada kedua mata, perut dan paha sejak lahir. Pasien ditemukan adanya ruam merah di pipi yang melintasi garis hidung yang berbentuk seperti kupu-kupu. Pasien lahir dari ibu lupus eritematosus sistemik yang didiagnosis SLE sejak tahun 2012 dan mendapat terapi hingga tahun 2014. Pada hari pertama masuk pasien tampak lesu, pada pemeriksaan fisik didapatkan memar (periorbital erythema) dan perdarahan subkonjungtiva pada kedua mata, memar. di perut dan paha, ruam kupu-kupu di pipi, ubun-ubun tidak menonjol. Hasil EKG berada dalam batas normal, dan ekokardiogram menunjukkan shunt kiri ke kanan karena paten foramen ovale (PFO) dan regurgitasi trikuspid ringan, keduanya tidak memerlukan perawatan jantung. Titer ANA IF 1:100 dengan pola berbintik, histon profil ANA positif, DNA anti-DS 60,7 IU/mL, dan C3 124,4 mg/dL. Pasien kemudian didiagnosis dengan lupus eritematosus neonatal ringan sampai sedang berdasarkan riwayat klinis, pemeriksaan fisik, dan pemeriksaan laboratorium.

Simpulan: Neonatal lupus erythematosus (NLE) bisa berakibat fatal sehingga observasi sangat penting.

References

  1. Bh Hon KL, Leung AKC. Neonatal lupus erythematosus. Hindawi Publishing Corporation-Autoimmune Diseases. 2012; 1-6
  2. Savino F, Viola S, Tarasco V et al. A neonatal lupus erythematosus: a cutaneous cases based update. Italian Journal of Pediatrics. 2016;42(1):1-5
  3. Ramphul K, Mejias SG, Ramphul-Sicharam Y. Cutaneous neonatal lupus erythematosus: A Case Report. Cureus. 2018;10(2): 1-4.
  4. Lee LA. Cutaneous lupus in infancy and childhood. Lupus. 2010;19( 9): 1112-7.
  5. Cheng CL, Galbraith S, Holland K. Congenital lupus erythematosus presenting at birth with widespread erosions, pancytopenia, and subsequent hepatobiliary disease. Pediatric Dermatology. 2010; 27(1)109-11.
  6. Silverman E, Jaeggi R. Non-cardiac manifestations of neonatal lupus erythematosus: Scandinavian Journal of Immunology. 2010; 72(3) 223-5.
  7. Hornberger LK, Rajaa NA. Spectrum of cardiac involvement in neonatal lupus. Scandinavian Journal of Immunology. 2010; 72(3);189-97.
  8. Wisuthsarewong W, Soongswang J, Chantor R. Neonatal lupus erythematosus: clinical character, investigation, and outcome. Pediatr Dermatol. 2011;28(2):115–21.
  9. Chacon RP, Ceballos LT, Cabrera RD, Perandones MT. Neonatal lupus erythematosus: A five-year case review. Reumatol Clin. 2014;10(3):170–1732. Available at www.reumatologiaclinica.org. Accessed on 2nd January 2022.
  10. Chhajed PS, Savaskar SV, Bhori NS, Bandichhode ST. Neonatal lupus erythematous. 2017; 4(4): 631-4
  11. A Karina Camillozzi Nogueira Freire1, Hannah Cade Guimarães1, Cristiane Aparecida Mendes1 et al. Neonatal lupus: case report with exuberant findings. Residência Pediátrica 2018;8(3):147-150.
  12. Brucato A, Cimaz R, Caporali R, Ramoni V, Buyon J. Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies. Clinical reviews in allergy & immunology. 2011;40(1): 27-41.
  13. Yang X. Clinical features, autoantibodies, and outcome of neonatal lupus erythematosus. Fetal and Pediatric Pathology.2020;1-6.
  14. Levy R, Briggs L, Silverman E, Pope E, Corrales IL. Cutaneous sequelae in neonatal lupus: a retrospective cohort study. Journal of the American Academy of Dermatology. 2019;1-20.
  15. Saxena A, Izmirly PM, Bomar RP, Golpanian R, Friedman D. Factors associated with long-term cardiac dysfunction in neonatal lupus. Ann Rheum Dis. 2019;0:1–8.

How to Cite

Desy, D. A., Wati, K. D. K. ., & Putra, P. J. . (2023). Neonatal lupus erythematosus: sebuah laporan kasus. Medicina, 54(2), 54–59. https://doi.org/10.15562/medicina.v54i2.1223

HTML
375

Total
101

Share

Search Panel