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与干燥综合征相关的肢端鱼鳞病样黏蛋白沉积症:一种特殊形式的胫前黏液性水肿?

Acral ichthyosiform mucinosis in association with Sjögren's syndrome: a peculiar form of pretibial myxedema?

作者信息

Yamazaki S, Katayama I, Satoh T, Yokozeki H, Nishioka K

机构信息

Department of Dermatology, Tokyo Medical and Dental University, School of Medicine, Japan.

出版信息

J Dermatol. 1993 Nov;20(11):715-8. doi: 10.1111/j.1346-8138.1993.tb01370.x.

DOI:10.1111/j.1346-8138.1993.tb01370.x
PMID:8300943
Abstract

Two Japanese women developed well-demarcated ichthyosiform plaques on the lateral aspect of their lower legs. Deposition of mucin was demonstrated throughout the papillary dermis, unlike the site of mucin deposition seen in pretibial myxedema. Their thyroid function was normal. The condition of both women was complicated by Sjögren's syndrome. One of them who presented with positive anti-microsomal and anti-thyroglobulin antibodies had goiter, suggesting that her malady was also complicated by Hashimoto's thyroiditis. Their skin manifestations differed from those described in cutaneous mucinosis including pretibial myxedema, specifically with regard to the well-demarcated ichthyosiform appearance, the mucin deposition in the papillary dermis, and the association with Sjögren's syndrome. To the best of our knowledge, our cases may thus be considered to be a previously undescribed form of cutaneous mucinosis associated with Sjögren's syndrome.

摘要

两名日本女性小腿外侧出现了边界清晰的鱼鳞病样斑块。整个乳头层真皮均有粘蛋白沉积,这与胫前黏液性水肿中所见的粘蛋白沉积部位不同。她们的甲状腺功能正常。两名女性的病情均合并干燥综合征。其中一名抗微粒体抗体和抗甲状腺球蛋白抗体呈阳性的女性患有甲状腺肿,提示其疾病还合并桥本甲状腺炎。她们的皮肤表现与包括胫前黏液性水肿在内的皮肤黏液瘤病中所描述的不同,特别是在边界清晰的鱼鳞病样外观、乳头层真皮中的粘蛋白沉积以及与干燥综合征的关联方面。据我们所知,我们的病例可能被认为是一种先前未描述过的与干燥综合征相关的皮肤黏液瘤病形式。

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