Paediatric morphoea: a holistic review. Part 2: diagnosis, measures of disease activity, management and natural history

Clin Exp Dermatol. 2020 Aug;45(6):679-684. doi: 10.1111/ced.14236. Epub 2020 May 24.

Abstract

Paediatric morphoea is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Defining optimum management strategies in paediatric morphoea remains an ongoing challenge, owing to the varied presentations and a relative paucity of paediatric-specific studies. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected and results analysed before summarizing them. In Part 1 of this review, we described the epidemiology, aetiopathogenesis and clinical classification; in this part, we discuss the diagnosis, markers of disease activity, management and natural history in paediatric morphoea.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Biomarkers
  • Child
  • Humans
  • Methotrexate / therapeutic use
  • Mycophenolic Acid / therapeutic use
  • Phototherapy*
  • Scleroderma, Localized / diagnosis*
  • Scleroderma, Localized / pathology
  • Scleroderma, Localized / therapy*
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / therapy

Substances

  • Adrenal Cortex Hormones
  • Biomarkers
  • Mycophenolic Acid
  • Methotrexate

Supplementary concepts

  • Juvenile-onset scleroderma