红细胞生成性原卟啉病

<正> 红细胞生成性原卟啉病(Erythropoietic protoporphyria简称EPP)于1961年由Magnus等首先报导,本病从国外报导来看发病率远比先天性红细胞卟啉病(EP)为多见,国内似尚未见于报导。本病皮损不严重,又无通常赖以诊断为卟啉病的卟啉尿,所以容易误

Erythropoietic Protoporphyria

The conditisn Known as Erythropoietic Protoporphia (EPP) is briefly reviewed in this paper and three cases reported. Among the three cases,case 2 (mother, 28yrs)and case3 (daughter, Tyts.) both had got sunlight senitivity since their ages of abo(?)t one. In addition, another 4 out of the 14 members of the family had similar Sunlight sensitivty, too。 Case 1 (3T yrs.), honever, had sunlight sensitivity only after the age of about 12 and ene of his children got similar Condition. In all those cases, the eruption involved the exposed areas, mainly the face and the dorsal sulface of the hands. The clinical manifestations and the laboratory findings of tbe three cases are discussed in this paper. It shows that the diagnosis of Epp may be made according to the cutaneous lesions caused by photosensitivity (especially the Late lesions), the positire family history, the absence of porphyrinuria and the histological findings。