Morphea in Childhood: An Update

Actas Dermosifiliogr (Engl Ed). 2018 May;109(4):312-322. doi: 10.1016/j.ad.2017.06.021. Epub 2017 Dec 14.
[Article in English, Spanish]

Abstract

Morphea is an inflammatory, fibrosing skin disorder. When it occurs in childhood, it is also known as localized juvenile scleroderma. It is more common in girls and typically appears around the age of 5 to 7 years. According to a recent classification system, morphea is divided into 5 types: circumscribed (plaque), linear, generalized, pansclerotic, and mixed. Approximately 40% of patients present extracutaneous manifestations. Childhood morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0.1%, methotrexate, topical or systemic corticosteroids, mycophenolate mofetil, bosentán, and topical imiquimod 5%. A variety of measuring tools are used to monitor response to treatment. Few prognostic studies have been conducted, but findings to date suggest that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae.

Keywords: Childhood; Esclerodermia juvenil localizada; Esclerodermia lineal; Facial hemiatrophy; Fototerapia; Hemiatrofia facial; Infancia; Linear scleroderma; Localized juvenile scleroderma; Methotrexate; Metotrexato; Morfea; Morphea; Phototherapy; Review literature as topic; Revisión narrativa.

Publication types

  • Review

MeSH terms

  • Acute-Phase Proteins / analysis
  • Bosentan / therapeutic use
  • Calcitriol / therapeutic use
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Imiquimod / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Incidence
  • Male
  • Phototherapy
  • Prevalence
  • Prognosis
  • Quality of Life
  • Scleroderma, Localized* / classification
  • Scleroderma, Localized* / epidemiology
  • Scleroderma, Localized* / pathology
  • Scleroderma, Localized* / therapy
  • Vitamin D / therapeutic use

Substances

  • Acute-Phase Proteins
  • Immunosuppressive Agents
  • Vitamin D
  • Calcitriol
  • Imiquimod
  • Bosentan