Localized scleroderma in a newborn (case report)

Abstract

Scleroderma is a rare condition in pediatrics although it ranks third (after juvenile idiopathic arthritis and systemic lupus erythematosus) among rheumatic diseases in children. Congenital scleroderma is considered casuistic since only single case reports are available in publications worldwide. The etiology of congenital scleroderma remains elusive. The principal pathogenic mechanisms are vascular network impairment, immune system dysfunction with abnormal regulation of cytokine release and altered collagen synthesis with fibroblast proliferation and fibrosis. This paper describes a newborn girl with congenital localized nodular scleroderma whose clinical presentations were detected at birth. The authors discuss difficulties with the diagnosis based on clinical and routine lab and instrumental tests. The role of modern morphological studies (given the lack of specific lab tests) to diagnose scleroderma in a newborn is emphasized. Finally, this paper provides a brief review of published data on congenital scleroderma. KEYWORDS: congenital scleroderma, children, diagnostics, differential diagnosis, morphological studies. FOR CITATION: Akhmina N.I., Utkin P.S., Shalatonin M.P. et al. Localized scleroderma in a newborn (case report). Russian Journal of Woman and Child Health. 2022;5(2):169–172 (in Russ.). DOI: 10.32364/2618-8430-2022-5-2-169-172.