Baudon J J, Fontaine J L, Larrègue M, Feldmann G, Laplane R
Arch Fr Pediatr. 1978 Jan;35(1):63-73.
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.
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。
