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家族性反应性穿通性胶原病

Familial reactive perforating collagenosis.

作者信息

Bhat Yasmeen J, Manzoor Sheikh, Qayoom Seema, Wani Roohi, Baba Asif Nazir, Bhat Arshad Hussian

机构信息

Department of Dermatology, STD & Leprosy, SKIMS Medical College Hospital, Srinagar, India.

出版信息

Indian J Dermatol. 2009;54(4):334-7. doi: 10.4103/0019-5154.57608.

DOI:10.4103/0019-5154.57608
PMID:20101333
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2807708/
Abstract

BACKGROUND

Reactive perforating collagenosis (RPC) is one of the rare forms of transepidermal elimination in which genetically altered collagen is extruded from the epidermis. This disease usually starts in early childhood as asymptomatic umbilicated papules on extremities, and the lesions become more conspicuous with age.

AIMS

The objective of our study was to determine the clinico-pathological features of RPC and the response to various treatment modalities.

METHODS

Ten patients of RPC, belonging to five different families, were studied clinically. Various laboratory investigations were carried out and diagnosis was made by histopathology of the lesions. Patients were given various topical and oral treatments.

RESULTS

RPC is familial in most cases without any definite inheritance pattern. It begins in childhood and the lesions are usually recurrent and become profuse and large with age. Systemic diseases have no role in the onset of lesions.

CONCLUSION

Oral and topical retinoids in combination with emollients is the best treatment option.

摘要

背景

反应性穿通性胶原病(RPC)是一种罕见的经表皮消除形式,其中基因改变的胶原蛋白从表皮挤出。这种疾病通常在儿童早期开始,表现为四肢无症状的脐状丘疹,随着年龄增长病变会更加明显。

目的

我们研究的目的是确定RPC的临床病理特征以及对各种治疗方式的反应。

方法

对来自五个不同家庭的10例RPC患者进行了临床研究。进行了各种实验室检查,并通过病变的组织病理学进行诊断。给予患者各种局部和口服治疗。

结果

大多数情况下,RPC是家族性的,没有任何明确的遗传模式。它始于儿童期,病变通常会复发,随着年龄增长会变得繁多且增大。全身性疾病在病变的发生中不起作用。

结论

口服和局部使用维甲酸联合润肤剂是最佳治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/a8aa3dd014b9/IJD-54-334-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/76936f0fc9b6/IJD-54-334-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/17d0e7b9a9e4/IJD-54-334-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/ac71af961e91/IJD-54-334-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/a8aa3dd014b9/IJD-54-334-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/76936f0fc9b6/IJD-54-334-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/17d0e7b9a9e4/IJD-54-334-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/ac71af961e91/IJD-54-334-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b366/2807708/a8aa3dd014b9/IJD-54-334-g004.jpg

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