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[A case of systemic sclerosis in childhood complicated by severe digital ulcers]
La Pediatria Medica E Chirurgica : Medical and Surgical Pediatrics
|September 1, 1984
Insights
Systemic sclerosis, a connective tissue disease, can affect children. This case highlights acrosclerosis with digital ulcers successfully treated by nifedipine.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Connective Tissue Diseases
Background:
- Systemic sclerosis is a rare, diffuse connective tissue disorder characterized by inflammation, fibrosis, and degeneration.
- It affects the skin and multiple internal organs, presenting a diagnostic and therapeutic challenge, especially in pediatric cases.
Observation:
- A six-year-old child diagnosed with systemic sclerosis, specifically the acrosclerosis subtype.
- The patient exhibited Raynaud's phenomenon and gastrointestinal involvement, common manifestations of the disease.
- Severe digital ulcerations developed on the hands and feet, significantly impacting quality of life.
Findings:
- The digital ulcerations, a severe complication of systemic sclerosis, showed significant improvement.
- Treatment with nifedipine, a calcium channel blocking agent, proved effective in healing these ulcerations.
Implications:
- This case underscores the importance of early recognition and management of systemic sclerosis in children.
- Nifedipine can be a valuable therapeutic option for managing severe digital ulcerations in pediatric patients with systemic sclerosis.
- Further research into pediatric systemic sclerosis is warranted to optimize treatment strategies.
Abstract:
Systemic sclerosis is a diffuse disorder of connective tissue in which there are inflammatory, fibrotic and degenerative changes in the skin and many internal organs. We describe a case of systemic sclerosis type "acrosclerosis" in a six years old child with Raynaud's phenomenon and gastrointestinal involvement. In the course of illness he presented severe digital ulcerations in the hands and in the feet, responsive to a calcium channel blocking agent nifedipine treatment.