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[Sclerodermiform porphyria cutanea tarda. Ultrastructural study].
Medicina Cutanea Ibero-Latino-Americana
|January 1, 1985
Summary
This study reports two cases of porphyria cutanea tarda (PCT) with scleroderma-like skin lesions. Treatment with urine alkalinization, phlebotomies, and N-acetyl-hydroxy-proline improved symptoms.
Area of Science:
- Dermatology
- Metabolic Disorders
Background:
- Porphyria cutanea tarda (PCT) is a metabolic disorder affecting approximately 30% of patients with scleroderma-like skin lesions.
- Understanding the clinical presentation and treatment of PCT is crucial for patient management.
Observation:
- Two cases of PCT with scleroderma-like lesions are presented.
- Patients were diabetic, non-alcoholic, and sun-tolerant.
- Skin lesions and porphyrin levels were monitored.
Findings:
- Porphyrin elimination decreased with urine alkalinization and phlebotomies.
- Scleroderma-like lesions showed improvement following administration of N-acetyl-hydroxy-proline.
Implications:
- This suggests a potential therapeutic role for N-acetyl-hydroxy-proline in managing PCT-associated skin manifestations.
- Further research may elucidate the mechanisms behind these treatment effects.