Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Skin and Immune Diseases, Beijing, China.
Department of Dermatology, Hospital Ramon y Cajal, Madrid, Spain.
Indian J Dermatol Venereol Leprol. 2020 Jul-Aug;86(4):359-365. doi: 10.4103/ijdvl.IJDVL_341_19.
Linear cutaneous lupus erythematosus is a rare subtype of lupus erythematosus (LE) that develops linear lesions following the lines of Blaschko. Linear cutaneous lupus erythematosus may present as various subtypes of LE, including linear discoid lupus erythematosus. There are few reports about pigmentedlinear discoid lupus erythematosus in the literature.
We aimed to summarize the clinical and pathological features of patients with pigmented linear discoid lupus erythematosus following the lines of Blaschko.
Eighteen patients with pigmented linear discoid lupus erythematosus attending the outpatient department of the Dermatology, Peking Union Medical College Hospital, China, were enrolled in the study. We recorded clinical data including sex, age at onset, disease duration, location and distribution of the lesions, symptoms, trigger factors, antinuclear antibody (ANA) testing, therapy, and therapeutic responses. Histopathological features were also summarized.
All 18 patients presented with well-defined brownish pigmented linear or segmental macules or plaques, following the lines of Blaschko. All the lesions were located on the head or neck. Unilaterally distributed lesions were found in 94.4% of patients. Two patients showed low titers of ANA in a speckled pattern. No systemic involvement or progression to systemic LE was noted. The patients were clinically diagnosed as pigmented lichen planus (55.6%), pigmented linear discoid lupus erythematosus (33.3%), and linear morphea (11.1%) before histopathological examination.
The study was retrospective and direct immunofluorescence was not performed. Not all patients' information was available and 4 patients were lost to follow-up because their contact information was changed.
Pigmented linear discoid lupus erythematosus mostly occurs on the head and neck. It manifests as brownish macules along the lines of Blaschko. Differentiation between pigmented linear discoid lupus erythematosus and other dermatoses that have a linear distribution can be difficult both clinically and pathologically, but histological details can help distinguish them.
线性皮肤红斑狼疮是一种罕见的红斑狼疮（LE）亚型，其沿着 Blaschko 线发展为线性病变。线性皮肤红斑狼疮可能表现为各种 LE 亚型，包括线性盘状红斑狼疮。文献中关于色素性线性盘状红斑狼疮的报道很少。
我们旨在总结沿着 Blaschko 线分布的色素性线性盘状红斑狼疮患者的临床和病理特征。
本研究纳入了 18 例在北京协和医院皮肤科就诊的色素性线性盘状红斑狼疮患者。我们记录了患者的临床数据，包括性别、发病年龄、病程、皮损部位和分布、症状、诱发因素、抗核抗体（ANA）检测、治疗及治疗反应。还总结了组织病理学特征。
18 例患者均表现为边界清楚的棕褐色色素性线性或节段性斑疹或斑块，沿 Blaschko 线分布。所有皮损均位于头颈部。94.4%的患者皮损单侧分布。2 例患者ANA 呈斑点型低滴度阳性。无系统性受累或进展为系统性 LE。在组织病理学检查前，患者临床诊断为色素性扁平苔藓（55.6%）、色素性线性盘状红斑狼疮（33.3%）和线状硬皮病（11.1%）。
本研究为回顾性研究，未进行直接免疫荧光检查。并非所有患者的信息都可用，由于联系方式发生变化，有 4 例患者失访。
色素性线性盘状红斑狼疮多发生于头颈部，表现为棕褐色斑疹，沿 Blaschko 线分布。在临床和病理上，色素性线性盘状红斑狼疮与具有线性分布的其他皮肤病难以区分，但组织学细节有助于鉴别。
