Cutaneous Neonatal Lupus Arising in an Infant Conceived From an Oocyte Donation Pregnancy | Dermatology | JAMA Dermatology | JAMA Network
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July 2016

Cutaneous Neonatal Lupus Arising in an Infant Conceived From an Oocyte Donation Pregnancy

Author Affiliations
  • 1Department of Dermatology, Stanford University School of Medicine, Palo Alto, California
  • 2Dermatology Medical Group of Oxnard and Camarillo, Camarillo, California
JAMA Dermatol. 2016;152(7):846-847. doi:10.1001/jamadermatol.2016.0001

As the pathogenesis behind neonatal lupus erythematosus (NLE) is thought to require both gestational factors, such as circulating maternal autoantibodies, and an underlying fetal genetic susceptibility,1 we report an informative case of cutaneous NLE involving an infant conceived through in vitro fertilization with a healthy oocyte donor and a gestational mother with Sjögren syndrome (SS), demonstrating that direct inheritance of genetic susceptibility from a mother afflicted with autoimmunity is not required for NLE.

Report of a Case

A 4-week-old otherwise healthy, full-term Asian American female infant presented with a 10-day history of annular, erythematous scaling plaques of the forehead and bilateral preauricular and postauricular regions (Figure 1). Notably, she was conceived via an unrelated donor oocyte and paternal sperm. Her gestational mother, who carried the patient to term, had a 10-year history of SS and was known to have tested positive for anti-Ro/SSA and anti-La/SSB antibodies. The father and the oocyte donor both reported no known history of autoimmune disease or other major medical problems. Given her gestational mother’s history of SS, the patient underwent an in utero fetal echocardiogram at 32 weeks’ gestation, and the results were normal. All other fetal ultrasonograms and an electrocardiogram obtained at birth were unremarkable.

Figure 1.  Cutaneous Neonatal Lupus
Cutaneous Neonatal Lupus

Clinical photograph of patient demonstrating characteristic annular, scaling plaque.

A punch biopsy of a facial lesion from the infant demonstrated vacuolar interface changes overlying a dense lymphohistiocytic infiltrate also containing numerous neutrophils (Figure 2). Numerous CD123-positive dendritic cells were also present within the infiltrate. Laboratory findings were positive for anti-Ro/SSA IgG and anti-La/SSB IgG; her antinuclear antibody titer was 1:80 with a coarse speckled pattern; and she demonstrated a mild transaminitis (aspartate transaminase [AST], 109 IU/L; alanine transaminase [ALT], 158 IU/L. Findings for the anti-U1RNP, anti-Smith, and anti–Jo-1 antibodies were negative. Findings of a repeat electrocardiogram, complete blood cell count, and thyroid studies were normal. As she continued to develop new lesions on the face and upper trunk over the next month, the patient was started on a treatment regimen of desonide, 0.05%, ointment for the face and triamcinolone, 0.1%, ointment for the trunk. Recommendations were provided regarding strict sun avoidance.

Figure 2.  Punch Biopsy Specimen From a Facial Lesion
Punch Biopsy Specimen From a Facial Lesion

A punch biopsy performed on a scaling plaque on the patient’s face at age 4 weeks reveals a vacuolar interface dermatitis overlying a dense dermal infiltrate composed of neutrophils, lymphocytes, and histiocytes, supporting the diagnosis of neonatal lupus (hematoxylin-eosin, original magnification ×200).

At age 8 weeks, the patient’s liver function test results peaked (AST, 518 IU/L; ALT, 648 IU/L; and alkaline phosphatase, 412 IU/L). These values subsequently down-trended and normalized by age 5 months. Skin examination at that time revealed only postinflammatory hyperpigmentation. By age 1 year, her skin lesions had resolved without scarring.


We describe the first case to our knowledge of cutaneous manifestations of NLE developing in an infant conceived from a healthy oocyte donor and born to a gestational mother with a history of autoimmune disease with circulating anti-Ro and anti-La antibodies. The pathogenesis of NLE is thought to involve a complex interplay between maternal factors such as circulating autoantibodies, the in utero environment, and underlying fetal genetic susceptibility.1-3 It has been difficult to assess the relative contributions of these factors because in nearly all cases, the infant is related to the affected mother. Our case involving the unique situation of NLE arising from an in vitro fertilization pregnancy suggests that while fetal genetic susceptibility may be required, direct transmission of these genes from the gestational mother is not.

We note a supportive report in the rheumatology literature describing an infant who developed heart block after birth to a surrogate mother with circulating anti-Ro/SSA antibodies, likely representing cardiac-predominant NLE.4 This case, in combination with the present patient’s classic cutaneous presentation, would indicate that the full spectrum of NLE’s manifestations can occur in the absence of autoimmunity in the maternal genetic donor. Therefore, from a clinical perspective, we suggest that NLE should be considered possible in an infant when there is a history of autoimmune connective tissue disease or appropriate circulating IgG autoantibodies in the gestational mother.

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Article Information

Corresponding Author: Ann L. Marqueling, MD, Department of Dermatology, Stanford University School of Medicine, 770 Welch Rd, Ste 261, Palo Alto, CA 94304 (

Published Online: March 9, 2016. doi:10.1001/jamadermatol.2016.0001.

Additional Contributions: We thank the patient’s parents for granting permission to publish this information. We also thank Mary Le, MD, for helpful discussion. Dr Le was not compensated for her contributions.

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