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获得性炎症性Blaschko线状皮肤病的临床和组织病理学特征

Clinical and Histopathological Characteristics of Acquired Inflammatory Blaschko-Linear Disorders.

作者信息

Mehta Nikhil, Khaitan Binod K, Ramam M, Bhari Neetu, Sethuraman Gomathy, Singh Manoj K

机构信息

Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Indian Dermatol Online J. 2024 Dec 11;16(1):116-122. doi: 10.4103/idoj.idoj_312_24. eCollection 2025 Jan-Feb.

Abstract

INTRODUCTION

Acquired inflammatory Blaschko-linear dermatoses have not been studied extensively. Descriptive studies on segmental vitiligo have yielded insights helpful in counseling patients. Similar insights are expected from studies on other acquired inflammatory Blaschko-linear diseases.

MATERIALS AND METHODS

Consecutive patients with an acquired inflammatory Blaschko-linear disease presenting to the dermatology outpatient department of the study center were recruited in a case series. Detailed history and examination, clinical photographs, and histopathological findings were recorded and analyzed. Features were compared between linear and generalized forms to look for any differences.

RESULTS

Out of 99 patients, linear lichen planus ( = 47), linear morphea ( = 31), and lichen striatus ( = 9) were observed most commonly. Skin lesions were present in multiple lines in 52 (52.5%). In 12 (12.1%), more than one anatomical site was involved. In 10 (10.1%), two different Blaschko-linear diseases were seen, and in 3 (3.1%) both diseases occurred in the same/adjacent segments. The disease extended from one or both ends in 64 (88.9%). Nineteen (19.2%) had both linear and generalized disease, with linear lesions being more severe than the generalized lesions ( = 0.038133). Some (18/47, 38.3%) linear lichen planus cases showed prominent atrophy since the onset and formed a distinct subset, predominantly over the head and neck site ( < 0.00001). Histopathology of linear lichen planus differed from controls with generalized lesions in terms of having deeper infiltrate ( = 0.000124), and multi-focal, rather than confluent, lichenoid infiltrates. Atrophy was noted from the onset in 13/31 (41.9%) cases of linear morphea.

LIMITATIONS

Limitations include cross-sectional design and lack of controls with generalized nonlinear diseases.

CONCLUSIONS

Acquired inflammatory Blaschko-linear disorders show distinct characteristics like involvement of multiple lines and sites, directional progression, and atrophic variants. These can be used for differentiating among different Blaschko-linear diseases, monitoring progression, and counseling patients.

摘要

引言

获得性炎症性线状皮肤病尚未得到广泛研究。对节段性白癜风的描述性研究为患者咨询提供了有益的见解。预计对其他获得性炎症性线状疾病的研究也会有类似的见解。

材料与方法

在一项病例系列研究中,招募了到研究中心皮肤科门诊就诊的连续的获得性炎症性线状疾病患者。记录并分析详细的病史、检查、临床照片和组织病理学结果。比较线状和泛发性形式的特征以寻找差异。

结果

在99例患者中,最常见的是线状扁平苔藓(n = 47)、线状硬皮病(n = 31)和线状苔藓(n = 9)。52例(52.5%)患者的皮肤病变呈多条线状分布。12例(12.1%)患者累及一个以上解剖部位。10例(10.1%)患者出现两种不同的线状皮肤病,3例(3.1%)患者两种疾病出现在同一/相邻节段。64例(88.9%)疾病从一端或两端扩展。19例(19.2%)患者既有线状疾病又有泛发性疾病,线状病变比泛发性病变更严重(P = 0.038133)。部分(18/47,38.3%)线状扁平苔藓病例自发病起就有明显萎缩,形成一个独特的亚组,主要分布在头颈部(P < 0.00001)。线状扁平苔藓的组织病理学与泛发性病变的对照组不同,表现为浸润更深(P = 0.000124),以及多灶性而非融合性的苔藓样浸润。13/31例(41.9%)线状硬皮病病例自发病起就有萎缩。

局限性

局限性包括横断面设计以及缺乏泛发性非线性疾病的对照。

结论

获得性炎症性线状疾病表现出明显的特征,如累及多条线和多个部位、定向进展以及萎缩性变体。这些特征可用于区分不同的线状皮肤病、监测病情进展以及为患者提供咨询。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7878/11753551/271a8cf20e33/IDOJ-16-116-g001.jpg

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