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疱疹样皮炎:病理生理学、临床表现、诊断与治疗

Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment.

作者信息

Clarindo Marcos Vinícius, Possebon Adriana Tomazzoni, Soligo Emylle Marlene, Uyeda Hirofumi, Ruaro Roseli Terezinha, Empinotti Julio Cesar

机构信息

West Paraná State University, Cascavel, PR, Brazil.

出版信息

An Bras Dermatol. 2014 Nov-Dec;89(6):865-75; quiz 876-7. doi: 10.1590/abd1806-4841.20142966.

DOI:10.1590/abd1806-4841.20142966
PMID:25387490
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4230654/
Abstract

Researches on DH have shown that it is not just a bullous skin disease, but a cutaneous-intestinal disorder caused by hypersensitivity to gluten. Exposure to gluten is the starting point of an inflammatory cascade capable of forming autoantibodies that are brought to the skin, where they are deposited, culminating in the formation of skin lesions. These lesions are vesico-bullous, pruritic, and localized especially on elbows, knees and buttocks, although atypical presentations can occur. Immunofluorescence of perilesional area is considered the gold standard for diagnosis, but serological tests help in cases where it is negative. Patients who follow gluten-free diets have better control of symptoms on the skin and intestine, as well as lower risks of progression to lymphoma. Dapsone remains the main drug for treatment, but it requires monitoring of possible side effects, some potentially lethal.

摘要

关于疱疹样皮炎(DH)的研究表明,它不仅是一种大疱性皮肤病,而且是一种由对面筋过敏引起的皮肤-肠道疾病。接触面筋是炎症级联反应的起点,这种反应能够形成自身抗体,这些抗体被带到皮肤并沉积在那里,最终导致皮肤病变的形成。这些病变为水疱-大疱性、瘙痒性,尤其好发于肘部、膝部和臀部,不过也可能出现非典型表现。病损周围区域的免疫荧光检查被认为是诊断的金标准,但在检查结果为阴性的情况下,血清学检测会有所帮助。遵循无麸质饮食的患者,其皮肤和肠道症状能得到更好的控制,进展为淋巴瘤的风险也更低。氨苯砜仍然是主要的治疗药物,但需要监测可能出现的副作用,其中一些可能是致命的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/1bfe750d6d46/abd-89-06-0865-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/1d606013c48d/abd-89-06-0865-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/720fc585ff94/abd-89-06-0865-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/8560bca33553/abd-89-06-0865-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/1bfe750d6d46/abd-89-06-0865-g04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/1d606013c48d/abd-89-06-0865-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/720fc585ff94/abd-89-06-0865-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/8560bca33553/abd-89-06-0865-g03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20ba/4230654/1bfe750d6d46/abd-89-06-0865-g04.jpg

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本文引用的文献

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Dermatitis herpetiformis: from the genetics to the development of skin lesions.疱疹样皮炎:从遗传学角度到皮肤病变的发展
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