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Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland

Ariane L Herrick1, Holly Ennis, Monica Bhushan

  • 1University of Manchester, Manchester, UK, and Salford Royal Hospital, Salford, UK. ariane.herrick@manchester.ac.uk

Arthritis Care & Research
|March 2, 2010
PubMed

Insights

Childhood scleroderma is rare, with localized forms more common than systemic sclerosis (SSc). This study determined incidence rates and patient demographics in the UK and Ireland.

Area of Science:

  • Pediatric rheumatology
  • Dermatology
  • Rare disease epidemiology

Background:

  • Childhood scleroderma is a rare and poorly understood group of conditions.
  • Accurate incidence data is crucial for understanding disease burden and resource allocation.

Purpose of the Study:

  • To determine the incidence of childhood scleroderma subtypes in the UK and Ireland.
  • To describe the demographic characteristics (age, sex, ethnicity) of affected children.

Main Methods:

  • A prospective study involving specialist medical associations (pediatricians, dermatologists, rheumatologists).
  • Reporting of all suspected cases of localized scleroderma or systemic sclerosis (SSc) in children under 16.
  • Data collection occurred between July 2005 and July 2007.

Main Results:

  • 94 valid cases confirmed from 185 notifications: 87 localized scleroderma (3.4/million/year) and 7 systemic sclerosis (SSc) (0.27/million/year).
  • Localized scleroderma predominantly affected females (63%) and white British children (82%), with a mean age of 10.4 years.
  • Systemic sclerosis cases were all female, predominantly white British (86%), with a mean age of 12.1 years. Median delay to consultation was significant for both forms.

Conclusions:

  • This study provides updated incidence estimates for childhood scleroderma and its subtypes in the UK and Ireland.
  • The findings highlight the rarity and demographic patterns of these conditions in children.
Abstract

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