[Morphea or juvenile localised scleroderma: Case report]

Rev Chil Pediatr. 2016 Jul-Aug;87(4):279-83. doi: 10.1016/j.rchipe.2016.01.013. Epub 2016 Mar 12.
[Article in Spanish]

Abstract

Introduction: Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues.

Objective: To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment.

Clinical case: The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling. The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS. She started with immunosuppressive therapy, physiotherapy, and occupational therapy.

Conclusions: We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected.

Keywords: Autoimmune; Autoinmune; Esclerodermia localizada juvenil; Juvenile localised scleroderma; Morfea; Morphea.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Delayed Diagnosis
  • Disease Progression
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Occupational Therapy / methods*
  • Physical Therapy Modalities*
  • Scleroderma, Localized / diagnosis*
  • Scleroderma, Localized / pathology
  • Scleroderma, Localized / therapy
  • Treatment Outcome

Substances

  • Immunosuppressive Agents