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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Nodular scleroderma in systemic sclerosis under D-penicillamine therapy.

T Sasaki1, K Denpo, H Ono

  • 1Department of Dermatology, Yokohama City University School of Medicine, Japan.

The Journal of Dermatology
|December 1, 1992
PubMed
Summary

Systemic sclerosis (SS) patients developing keloidal lesions during D-penicillamine (DPC) therapy may experience fibroblast activation. This suggests a complex interaction between DPC

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Area of Science:

  • Dermatology and rheumatology, focusing on connective tissue diseases and drug-induced reactions.

Background:

  • Systemic sclerosis (SS) is a chronic autoimmune disease characterized by skin and organ fibrosis.
  • D-penicillamine (DPC) is a medication used to treat systemic sclerosis, aiming to reduce fibrosis.

Observation:

  • A 36-year-old woman with rapidly progressing systemic sclerosis, including lung and esophageal involvement, was treated with DPC starting at age 38.
  • While overall skin sclerosis showed some improvement, keloidal nodules developed on her upper chest at age 44.

Findings:

  • The development of keloidal lesions (nodular scleroderma) occurred during DPC therapy.
  • No other adverse reactions to DPC were identified, suggesting a localized phenomenon.
  • Fibroblast activation in the keloidal lesions is hypothesized despite DPC's generally suppressive effects.

Implications:

  • This case highlights a potential, albeit rare, paradoxical reaction to DPC therapy in systemic sclerosis.
  • Further research may elucidate the mechanisms of fibroblast activation in keloid formation during immunosuppressive therapy.
  • Understanding such reactions is crucial for optimizing treatment strategies in systemic sclerosis.