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迟发性皮肤卟啉症伴硬皮病、鱼鳞病、脱发和白癜风。

Porphyria Cutanea Tarda Presenting with Scleroderma, Ichthyosis, Alopecia, and Vitiligo.

作者信息

MacGillivray Megan E, Salopek Thomas G

机构信息

Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.

出版信息

Case Rep Dermatol. 2018 May 17;10(2):115-121. doi: 10.1159/000488899. eCollection 2018 May-Aug.

DOI:10.1159/000488899
PMID:29928199
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6006631/
Abstract

Porphyria cutanea tarda (PCT) is a cutaneous porphyria that presents later in life with cutaneous findings in sun-exposed sites. We report a complex case of PCT in a 67-year-old woman with an unusual constellation of cutaneous findings: scleroderma, acquired ichthyosis, and nonscarring alopecia. Possible triggers for her PCT include tamoxifen treatment for breast cancer and carrier status of the hemochromatosis gene. High-dose chloroquine was used to successfully achieve clinical remission and normalize her uroporphyrins. While on chloroquine she developed extensive classic vitiligo. It is not clear if this is another feature of her complex and unusual PCT, or a consequence of her antimalarial therapy.

摘要

迟发性皮肤卟啉症(PCT)是一种皮肤卟啉症,在生命后期出现,在阳光暴露部位有皮肤表现。我们报告了一例67岁女性的复杂PCT病例,其具有一系列不寻常的皮肤表现:硬皮病、获得性鱼鳞病和非瘢痕性脱发。她患PCT的可能诱因包括乳腺癌的他莫昔芬治疗和血色素沉着症基因的携带者状态。高剂量氯喹成功实现了临床缓解并使她的尿卟啉水平恢复正常。在服用氯喹期间,她出现了广泛的典型白癜风。尚不清楚这是她复杂且不寻常的PCT的另一特征,还是抗疟治疗的结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31d1/6006631/ef3c490834c5/cde-0010-0115-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31d1/6006631/6ae877c8b9cd/cde-0010-0115-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31d1/6006631/8516b410a753/cde-0010-0115-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31d1/6006631/ef3c490834c5/cde-0010-0115-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31d1/6006631/6ae877c8b9cd/cde-0010-0115-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31d1/6006631/8516b410a753/cde-0010-0115-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31d1/6006631/ef3c490834c5/cde-0010-0115-g03.jpg

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引用本文的文献

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本文引用的文献

1
Porphyria cutanea tarda: an intriguing genetic disease and marker.迟发性皮肤卟啉症:一种引人入胜的遗传性疾病及标志物。
Int J Dermatol. 2017 Jun;56(6):e106-e117. doi: 10.1111/ijd.13580. Epub 2017 Mar 21.
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Cutaneous paraneoplasia.皮肤副肿瘤综合征。
Clin Dermatol. 2011 Sep-Oct;29(5):541-7. doi: 10.1016/j.clindermatol.2010.09.022.
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Porphyria cutanea tarda induced by tamoxifen.他莫昔芬诱发的迟发性皮肤卟啉病。
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Ichthyotic-appearing skin changes associated with childhood morphea, systemic sclerosis, and systemic lupus erythematosus/scleroderma overlap.与儿童硬斑病、系统性硬化症以及系统性红斑狼疮/硬皮病重叠综合征相关的鱼鳞病样皮肤改变。
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Familial and sporadic porphyria cutanea tarda: clinical and biochemical features and risk factors in 152 patients.家族性和散发性迟发性皮肤卟啉症:152例患者的临床、生化特征及危险因素
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Associations among behavior-related susceptibility factors in porphyria cutanea tarda.迟发性皮肤卟啉病相关易感性因素之间的关联。
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Sclerodermatous changes of face, neck and scalp associated with familial porphyria cutanea tarda.面部、颈部和头皮的硬皮病样改变与家族性迟发性皮肤卟啉症相关。
Clin Exp Dermatol. 2008 Jul;33(4):422-4. doi: 10.1111/j.1365-2230.2007.02579.x. Epub 2008 May 21.
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Scleroderma-like disorders.硬皮病样疾病
Autoimmun Rev. 2008 Feb;7(4):331-9. doi: 10.1016/j.autrev.2007.12.004. Epub 2008 Jan 11.
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Acquired ichthyosis.获得性鱼鳞病
J Am Acad Dermatol. 2006 Oct;55(4):647-56. doi: 10.1016/j.jaad.2006.04.047.
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Acquired ichthyosis associated with an overlap syndrome of systemic sclerosis and systemic lupus erythematosus.获得性鱼鳞病与系统性硬化症和系统性红斑狼疮重叠综合征相关。
J Dermatol. 2006 Jan;33(1):52-4. doi: 10.1111/j.1346-8138.2006.00010.x.