Chinese Journal of Dermatology ›› 1990, Vol. 23 ›› Issue (1): 8-8.

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CLINICAL AND PORPHYRIN ANALYSIS ON 38 PATIENTS WITH ERYTHROPOIETIC PROTOPORPHYRIA (EPP)

LIAO Kang-Huang, ZHENG Pei-Shu   

  1. Institute of Dermatolagy, Shanghai Medical University
  • Received:1988-07-29 Revised:2008-10-22 Online:1990-02-15 Published:1990-02-15

Abstract: The clinical and porphyrin study on 38 cases of EPP arc presented.It consists of 62.5% porphrias we have seen.The average ale at onset is 6.8 years which is higher than that reported in other countries.Sunlight was the raajor precipitating factor.However,55.3% patients believed that strong wind could also provoke the onset of the disease.In acute attack,patients had sensation of burning and stinging with erythematous edema on exposed skin,followed by linear and/or vermicular pits and scars,pebble stona-like thickening of the skin on the dorsa of the hands,waxy-thickening of the facial skin with orange peel-like nose,radial grooves and scarring of the lips and perioral skin.The diagnosis was confirmed by these characteristic findings as well as elevated levels of protoporphyrin in the bland and negarive porphyrins in the urine.Fluorescence imcroscopy for RSV protoporphyrin proved to be useful as a rapid and reliable method.