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Progressive systemic sclerosis in a child

Arun K De1, Kallol Das, Archan Sil

  • 1Department of Pediatric Medicine, Medical College, Kolkata, India.

Indian Journal of Dermatology
|October 2, 2013
PubMed

Insights

Systemic sclerosis is rare in children. This case study details an 11-year-old girl with progressive systemic sclerosis, highlighting its varied symptoms in pediatric patients.

Area of Science:

  • Rheumatology
  • Pediatrics
  • Dermatology

Background:

  • Systemic sclerosis is a rare, heterogeneous autoimmune disease affecting connective tissues.
  • Pediatric cases of systemic sclerosis are uncommon, representing less than 10% of all diagnoses.

Purpose of the Study:

  • To report a rare case of progressive systemic sclerosis in an 11-year-old girl.
  • To illustrate the diverse clinical manifestations of pediatric systemic sclerosis.

Main Methods:

  • Case report of an 11-year-old female patient.
  • Clinical assessment and documentation of presenting symptoms.

Main Results:

  • The patient presented with cutaneous sclerosis, microstomia, mask-like facies, sclerodactyly, esophageal dysmotility, Raynaud's phenomenon, arthralgia, and pulmonary fibrosis.
  • The case exemplifies the multi-system involvement characteristic of progressive systemic sclerosis.

Conclusions:

  • Systemic sclerosis, though rare, can present with significant multi-system involvement in children.
  • Early recognition and comprehensive management are crucial for pediatric patients diagnosed with systemic sclerosis.

Keywords:
MicrostomiaRaynaud's phenomenonprogressive systemic sclerosispulmonary fibrosissclerodactyly

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