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[肠病性肢端皮炎。两例家族性病例应用硫酸锌治疗的解剖学与临床研究]

[Acrodermatitis enteropathica. Anatomo-clinical study of 2 familial cases treated with zinc sulfate].

作者信息

Baudon J J, Fontaine J L, Larrègue M, Feldmann G, Laplane R

出版信息

Arch Fr Pediatr. 1978 Jan;35(1):63-73.

PMID:637663
Abstract

Two familial cases of acrodermatitis enteropathica began at the time of weaning, with characteristic dermatologic lesions and digestive signs, resulting in lack of weight gain: at 13 months of age growth future was evident in the elder child. The anatomic lesions were those of an exophagitis and, in the duodenal mucosa, a dilation of the capillaries of the chorion in the absence of villous atrophy. The ultrastructural study showed inclusions in Paneth's cells, and changes in keratin cells, with intra-cytoplasmic vacuoles and abnormal melanin pigment in the skin. The administration of zinc sulphate induced a rapid recovery of cutaneous lesions and resumption of growth. The interruption of treatment resulted twice in a quite immediate relapse of cutaneous signs in the first patient, which was rapidly reversible with the reintroduction of zinc sulphate.

摘要

两例家族性肠病性肢端皮炎病例始于断奶期,伴有特征性皮肤病变和消化系统症状,导致体重不增:年长患儿13个月大时生长发育迟缓明显。解剖学病变表现为食管炎,十二指肠黏膜中绒毛膜毛细血管扩张,无绒毛萎缩。超微结构研究显示潘氏细胞中有包涵体,角质形成细胞有变化,皮肤中有胞质内空泡和异常黑色素沉着。硫酸锌治疗使皮肤病变迅速恢复,生长发育重新开始。第一位患者中断治疗后皮肤症状两次立即复发,重新使用硫酸锌后症状迅速逆转。

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