[Sclerodermiform porphyria cutanea tarda. Ultrastructural study].
Approximately 30% of patients affected of PCT present scleroderma-like lesion of the skin. Two cases of PCT, presenting scleroderma-like lesions are reported. The patients were diabetic but not alcoholic and tolerate relatively well sunshine. Porphyrin elimination diminished with urine alkalinization and phlebotomies, and the scleroderma-like lesions improved with N-acetyl-hydroxy-proline administration.
D000368:Aged; D000818:Animals; D001639:Bicarbonates / Q000627:therapeutic use; D006224:Cricetinae; D048909:Diabetes Complications; D003937:Diagnosis, Differential; D006470:Hemorrhage; D006801:Humans; D006909:Hydroxyproline / Q000031:analogs & derivatives / Q000627:therapeutic use; D007249:Inflammation; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D012595:Scleroderma, Systemic / Q000175:diagnosis*; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D012964:Sodium / Q000627:therapeutic use; D017693:Sodium Bicarbonate
D001639:Bicarbonates; D017693:Sodium Bicarbonate; D012964:Sodium; C012393:oxaceprol; D006909:Hydroxyproline
Porfiria cutánea tarda esclerodermiforme. Estudio ultraestructural.