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[获得性反应性穿通性皮病。2例患者使用别嘌醇治疗成功]

[Acquired reactive perforating dermatosis. Successful treatment with allopurinol in 2 cases].

作者信息

Krüger K, Tebbe B, Krengel S, Goerdt S, Orfanos C E

机构信息

Klinik und Poliklinik für Dermatologie, Universitätsklinikum Benjamin Franklin, Hindenburgdamm, Berlin.

出版信息

Hautarzt. 1999 Feb;50(2):115-20. doi: 10.1007/s001050050874.

Abstract

Perforating disorders represent a heterogenous group of dermatoses characterized by transepithelial elimination of dermal structures. Primary perforating disorders should be distinguished from secondary perforating disorders in which perforation with transepithelial elimination is a rare component of a variety of dermatoses. The primary perforating disorders are hyperkeratosis follicularis et parafollicularis in cutem penetrans (Kyrle's disease), elastosis perforans serpiginosa and perforating folliculitis. Acquired reactive perforating dermatosis (also known as acquired reactive perforating collagenosis) together with the hereditary variant of the reactive perforating collagenosis represent further examples of the primary perforating disorders. We report on 84 year old and 96 year old female patients with an acquired perforating dermatosis. Both of the patients additionally showed diabetes and hyperuricemia. Oral administration of allopurinol (100 mg daily) led to a healing of the disseminated skin lesions in 1-2 weeks. After a follow-up period of 6 months, both patients were in complete remission. On one hand, these results prove again the existence and the severity of this disease, and on the other hand suggest an immunomodulating or differentiation-promoting action in addition to the uricostatic effect of allopurinol.

摘要

穿通性疾病是一组异质性皮肤病,其特征是真皮结构经上皮排出。原发性穿通性疾病应与继发性穿通性疾病相鉴别,在继发性穿通性疾病中,经上皮排出的穿通现象是多种皮肤病中罕见的组成部分。原发性穿通性疾病包括穿通性毛囊和毛囊旁角化过度(凯尔氏病)、匐行性穿通性弹力纤维病和穿通性毛囊炎。获得性反应性穿通性皮肤病(也称为获得性反应性穿通性胶原病)以及反应性穿通性胶原病的遗传性变异代表了原发性穿通性疾病的其他例子。我们报告了两名患有获得性穿通性皮肤病的女性患者,年龄分别为84岁和96岁。两名患者均还患有糖尿病和高尿酸血症。口服别嘌醇(每日100毫克)导致播散性皮肤病变在1至2周内愈合。经过6个月的随访期,两名患者均完全缓解。一方面,这些结果再次证明了这种疾病的存在及其严重性,另一方面表明别嘌醇除了具有尿酸抑制作用外,还具有免疫调节或促进分化的作用。

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