Chanprapaph Kumutnart, Tankunakorn Jutamas, Suchonwanit Poonkiat, Rutnin Suthinee
Division of Dermatology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Dermatol Ther (Heidelb). 2021 Feb;11(1):131-147. doi: 10.1007/s13555-020-00471-y. Epub 2020 Dec 5.
Cutaneous manifestations are central to the primary diagnosis of systemic lupus erythematosus (SLE). However, information on the clinical, histopathologic, and direct immunofluorescence (DIF) features among subtypes of cutaneous lupus erythematosus (CLE), as well as longitudinal prospective observational study to evaluate the natural history and the progression to SLE, is lacking among Asians. Our objectives are to summarize the differences in the clinical, histopathologic, and DIF characteristics and serological profiles between various subtypes of CLE, and to provide its natural history and the association with disease activity in our Asian population.
A prospective observational study on CLE patients was performed between May 2016 and May 2020. Patients underwent full physical/dermatologic examination, skin biopsy for histology, and DIF. Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores and laboratory data were evaluated. Time schedule and characteristics for resolution and/or the disease progression to SLE were recorded in subsequent follow-ups.
Of 101 biopsy-proven CLE patients, 25 had acute CLE (ACLE), 8 had subacute CLE (SCLE), 39 had chronic CLE (CCLE) only, 22 had CCLE with SLE, and 7 had LE-nonspecific cutaneous lesions only. Patients with exclusive CLE showed lower female preponderance, serological abnormalities, and correlation to systemic disease. However, when CLE was accompanied with any LE-nonspecific cutaneous manifestations, they were associated with high antinuclear antibody (ANA) titer, renal, hematologic, joint involvement, and greater SLEDAI score. Of 207 biopsy sections, SCLE/CCLE regardless of systemic involvement showed significantly higher percentage of superficial/deep perivascular and perieccrine infiltration than ACLE. On DIF, deposition of multiple immunoreactants was associated with higher systemic disease. Approximately 10% of CLE-only patients later developed SLE but had mild systemic involvement.
Our findings support that each CLE subtype has a diverse and unique character. Comprehensive understanding of the differences among CLE subtypes is important for achieving the correct diagnosis and providing appropriate disease monitoring and management.
皮肤表现是系统性红斑狼疮（SLE）初步诊断的核心。然而，亚洲人群缺乏关于皮肤型红斑狼疮（CLE）各亚型的临床、组织病理学和直接免疫荧光（DIF）特征的信息，以及评估其自然史和向SLE进展情况的纵向前瞻性观察研究。我们的目的是总结CLE各亚型在临床、组织病理学、DIF特征和血清学方面的差异，并提供其在亚洲人群中的自然史及其与疾病活动的关联。
2016年5月至2020年5月对CLE患者进行了一项前瞻性观察研究。患者接受了全面的体格/皮肤科检查、皮肤活检以进行组织学检查和DIF检查。评估了系统性红斑狼疮疾病活动指数2000（SLEDAI-2K）评分和实验室数据。在随后的随访中记录了缓解和/或疾病进展为SLE的时间安排和特征。
在101例经活检证实的CLE患者中，25例为急性CLE（ACLE），8例为亚急性CLE（SCLE），39例仅为慢性CLE（CCLE），22例CCLE合并SLE，7例仅为LE非特异性皮肤病变。单纯CLE患者的女性优势、血清学异常及与全身疾病的相关性较低。然而，当CLE伴有任何LE非特异性皮肤表现时，它们与高抗核抗体（ANA）滴度、肾脏、血液学、关节受累及更高的SLEDAI评分相关。在207个活检切片中，无论是否有全身受累，SCLE/CCLE的浅表/深部血管周围和汗腺周围浸润百分比均显著高于ACLE。在DIF方面，多种免疫反应物的沉积与更高的全身疾病相关。约10%的单纯CLE患者后来发展为SLE，但全身受累较轻。
我们的研究结果支持每种CLE亚型都具有多样且独特的特征。全面了解CLE各亚型之间的差异对于实现正确诊断以及提供适当的疾病监测和管理非常重要。
