Abstract
Objective. Cutaneous neonatal lupus resembles subacute cutaneous lupus erythematosus (SCLE), and photosensitivity is a common symptom. Tumor necrosis factor α (TNFα) release by ultraviolet light-exposed keratinocytes may be exaggerated in SCLE patients who have the haplotype TNFα -308A;DRB1*03. Accordingly, this study was undertaken to seek genetic and histologic evidence for a role of TNFα in the pathogenesis of cutaneous neonatal lupus. Methods. DNA was isolated from 83 children (22 with rash, 35 with congenital heart block [CHB], 26 unaffected siblings) and 58 mothers from the Research Registry for Neonatal Lupus. Results. The -308A allele (associated with higher TNFα production), HLA-DRQB1*02, and HLA-DRB1*03 were each present in the majority of children with rash (64%, 68%, and 64%, respectively). The frequency of all 3 6p alleles occurring together in 1 individual was greater in children with rash than in children who had either CHB or no manifestation of neonatal lupus (59% versus 30%; P = 0.02). This association with neonatal lupus rash was equivalent to published findings in a cohort of patients with SCLE, but significantly greater than the association in patients with discoid lupus erythematosus. Prominent TNFα staining in the epidermis was observed in lesional skin from 3 children with rash, but not in skin from a healthy neonate. Conclusion. Taken together, the finding of a genetic predisposition to generate increased levels of TNFα following tissue injury and the histologic demonstration of TNFα in the target organ support the notion that this inflammatory cytokine plays a role in the pathogenesis of cutaneous neonatal lupus. Furthermore, the results of these studies provide evidence of a biologic link between neonatal lupus and the rash of SCLE.
Original language | English (US) |
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Pages (from-to) | 2598-2603 |
Number of pages | 6 |
Journal | Arthritis and Rheumatism |
Volume | 50 |
Issue number | 8 |
DOIs | |
State | Published - Aug 2004 |
ASJC Scopus subject areas
- Immunology and Allergy
- Rheumatology
- Immunology
- Pharmacology (medical)