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播散性汗孔角化症伴特发性血小板减少症——汗孔角化症及相关疾病的病例报告与文献综述

Disseminated Porokeratosis with Idiopathic Thrombocytopenia - Case Report and Literature Review of Porokeratosis and Related Disorders.

作者信息

Chokoeva Anastasiya Atanasova, Wollina Uwe, Lotti Torello, Maximov Georgi Konstantinov, Lozev Ilia, Tchernev Georgi

机构信息

Medical University Plovdiv, Dermatology and Venereology, Vasil Aprilov 15A, Plovdiv 4000, Bulgaria.

Städtisches Klinikum Dresden - Department of Dermatology and Allergology, Dresden, Sachsen, Germany.

出版信息

Open Access Maced J Med Sci. 2018 Jan 13;6(1):139-142. doi: 10.3889/oamjms.2018.017. eCollection 2018 Jan 25.

Abstract

BACKGROUND

Porokeratosis is characterised by one or more atrophic patches surrounded by a distinctive peripheral keratotic ridge, typically found on sun-exposed areas, with several clinical variants and typical histological findings. Despite ultraviolet radiation, varies antibody - related autoimmune disease treated with systemic steroids and other immunosuppressive conditions such as chronic liver disease, HIV and organ transplantations have been implicated in its etiopathology.

CASE REPORT

We present a case of porokeratosis, associated with idiopathic thrombocytopenia in 74 - year old, otherwise healthy male patient, as we discuss the previously reported associated disorders.

CONCLUSION

Regarding all of the polymorphism of clinical presentation, associated disorders and treatment responses, we could conclude that disseminated porokeratosis is still an unknown well disorder, which will continue to surprise the physicians in future. The screening and follow up of the patients is mandatory in all cases because as we see, porokeratosis has multiple unexpected faces, which require circumstantial clinical and paraclinical behaviour.

摘要

背景

汗孔角化症的特征是一个或多个萎缩性斑块被独特的周边角化性隆起所环绕,通常见于阳光暴露部位,有多种临床变体和典型的组织学表现。尽管紫外线辐射与之相关,但多种自身抗体相关的自身免疫性疾病、接受全身类固醇治疗的疾病以及其他免疫抑制状况(如慢性肝病、HIV感染和器官移植)也被认为与其发病机制有关。

病例报告

我们报告一例74岁、其他方面健康的男性汗孔角化症患者,该病例与特发性血小板减少症相关,并讨论先前报道的相关疾病。

结论

鉴于临床表现、相关疾病及治疗反应的所有多态性,我们可以得出结论,播散性汗孔角化症仍是一种未知的疑难病症,未来仍会让医生感到惊讶。在所有病例中对患者进行筛查和随访是必要的,因为如我们所见,汗孔角化症有多种意想不到的表现,需要根据具体情况进行临床和辅助检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5867/5816286/f538e15ff0fe/OAMJMS-6-139-g001.jpg

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