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Progressive systemic sclerosis in a child: case report

C C Tai1, P Lee, R E Wood

  • 1Department of Dentistry, Ontario Cancer Institute incorporating Princess Margaret Hospital, Canada.

Pediatric Dentistry
|July 1, 1993
PubMed
Summary
This summary is machine-generated.

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This case report details pediatric progressive systemic sclerosis, highlighting its significant systemic and oral manifestations. Early recognition of these symptoms is crucial for managing this rare autoimmune condition.

Area of Science:

  • Rheumatology
  • Pediatric Autoimmunology
  • Dental Medicine

Background:

  • Progressive systemic sclerosis (PSS) is a rare autoimmune disease characterized by ত্বক thickening and internal organ fibrosis.
  • Pediatric PSS presents unique challenges in diagnosis and management due to its rarity and potential for widespread systemic involvement.

Observation:

  • A case of pediatric PSS is presented, detailing its systemic features such as sclerodactyly, Raynaud's phenomenon, and organ-specific manifestations.
  • Oral manifestations observed include reduced interincisal distance, xerostomia, mandibular bone resorption, and advanced periodontal disease.

Findings:

  • Systemic findings encompass sclerodactyly, Raynaud's phenomenon, telangiectasia, calcinosis, myositis, arthritis, tenosynovitis, renal failure, esophageal hypomotility, pulmonary fibrosis, and heart failure.

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  • Dental findings include xerostomia, telangiectasia, increased periodontal ligament width, mandibular osseous resorption, periodontal disease, and a high DMFT (decayed, missing, and filled teeth) score.

    • Implications:

    • The diverse systemic and oral manifestations necessitate a multidisciplinary approach for comprehensive patient care.
    • Prognosis is variable, ranging from spontaneous remission to life-threatening visceral compromise, underscoring the need for vigilant monitoring and timely intervention.