The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus,☆☆,

https://doi.org/10.1016/S0190-9622(99)70146-5Get rights and content

Abstract

Background: Cutaneous neonatal lupus erythematosus (NLE) is an uncommon disease described mainly through isolated case reports. Objective: Our purpose was to examine the cutaneous spectrum, clinical associations, and course of disease in babies with anti-Ro-positive NLE. Methods: This is a retrospective case series evaluation of newborns with anti-Ro-positive NLE seen at a single ambulatory care university center over a 20-year period. Cases were drawn from a population of 3.2 million. Follow-up was at least 3 years. Results: Four boys and 14 girls were included in our evaluation. Distribution of skin lesions in 18 babies was as follows: face, 17; periorbital “owl-eye” or “eye mask” facial rash, 14; scalp, 15; arms and legs, 13; trunk and groin, 6. Crusted lesions were predominant in 3. Photosensitivity was seen in 12, and features of cutis marmorata telangiectasia congenita were observed in 4. In 17 neonatal lupus was not suspected until the dermatology consultation. Noncutaneous manifestations included thrombocytopenia in 4, cholestatic hepatitis in 3, and congenital heart block in 3. Four patients had residual telangiectasia that persisted for 3 or more years but eventually cleared in 2 patients. Three babies had dyspigmentation that spontaneously cleared within 22 months. None had atrophy or scarring. Conclusion: Periorbital, scalp, and extremity lesions are common in cutaneous NLE. Crusted lesions predominated in male infants. In children selected by cutaneous involvement, thrombocytopenia and hepatic disease were present as frequently as cardiac disease and occurred more frequently in male babies with crusted skin lesions. Children with cutaneous NLE should be evaluated for hematologic and hepatic as well as cardiac involvement.(J Am Acad Dermatol 1999;40:675-81.)

Section snippets

SUBJECTS AND METHODS

All 18 babies were seen within the first 2 months of life by one of the authors at the University of Colorado Health Sciences Center. All 18 babies and their mothers had anti-Ro (anti-SS-A) autoantibodies in their sera. A retrospective chart analysis was undertaken and examined for age at onset, distribution and type of skin lesions, history of sun-induced lesions, duration of lesions, residual lesions on follow-up examinations at least 3 years later, and presence of extracutaneous involvement.

RESULTS

This group comprised 14 female and 4 male infants. Fourteen were white, 2 black, 1 Hispanic, and 1 Asian. This is similar to the racial demographics of Colorado. Facial lesions were observed in 17 of 18 with the characteristic periorbital “owl-eye” or “eye mask” facial rash (Fig 1) seen in 14.

. Periorbital “owl-eye” or “eye mask” appearance characteristic of cutaneous NLE.

Involvement of the scalp was observed in 15, of the arms and legs in 13, and of the trunk and groin in 6. Crusted lesions

DISCUSSION

Although neonatal lupus is being increasingly recognized, in virtually none of the babies in this series was the diagnosis of NLE initially suspected. The characteristic periorbital “owl-eye” appearance was present in the majority of babies and we believe should be strongly emphasized as a cutaneous clue to NLE. However, the absence of the distinctive facial rash does not exclude the diagnosis of NLE. We observed thickly crusted facial and extremity lesions in 3 babies who did not have this

References (35)

  • EC Vonderheid et al.

    Neonatal lupus erythematosus

    Arch Dermatol

    (1976)
  • RM Watson et al.

    Neonatal lupus erythematosus: a clinical, serological and immunogenetic study with review of the literature

    Medicine

    (1984)
  • LA Lee et al.

    New clinical and laboratory findings in neonatal lupus

    Am J Dis Child

    (1984)
  • LA Lee et al.

    Neonatal lupus erythematosus

    Semin Dermatol

    (1988)
  • MB Hetem et al.

    Neonatal lupus erythematosus

    Int J Dermatol

    (1996)
  • S Miyagawa et al.

    Placental transfer of anticytoplasmic antibodies in annular erythema of newborns

    Arch Dermatol

    (1981)
  • A Kettler et al.

    Annular eruptions of infancy and neonatal lupus erythematosus

    Arch Dermatol

    (1987)
  • Cited by (115)

    • Cutaneous sequelae in neonatal lupus: A retrospective cohort study

      2020, Journal of the American Academy of Dermatology
      Citation Excerpt :

      Atrophic scarring is the most worrisome complication of CNLE given the higher likelihood of persistence and lack of effective therapies available. Scarring is scarcely reported in CNLE; Weston et al7 found no atrophy or scarring developed in their 18 patients. Neiman et al,9 however, found that 10 of 51 patients had pitting, scarring, or atrophy after a minimum 2 years of follow-up.

    • Polymorphism and differential diagnosis of neonatal lupus erythematosus

      2020, Annales de Dermatologie et de Venereologie
    • Neonatal Lupus Erythematosus

      2015, Textbook of Pediatric Rheumatology
    View all citing articles on Scopus

    Supported by grant R03 DK 53754 from the National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health.

    ☆☆

    Reprints are not available from the authors.

    0190-9622/99/$8.00 + 0   16/1/96584

    View full text