Department of Dermatology, Hospital Pulau Pinang, Penang, Malaysia.
Rheumatology Unit, Department of Medicine, Hospital Pulau Pinang, Penang, Malaysia.
Clin Exp Dermatol. 2022 Aug;47(8):1490-1501. doi: 10.1111/ced.15190. Epub 2022 May 23.
BACKGROUND: Cutaneous lupus erythematosus (CLE) is a chronic, autoimmune skin disease with a wide spectrum of clinical presentations in different populations. AIM: To study the clinicohistological and immunological features of CLE in a multiethnic population and to identify the predictive factors of disease severity based on the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). METHODS: This was a cross-sectional study of CLE conducted from March 2019 to February 2020. RESULTS: In total, 111 patients were recruited with a female/male ratio of 4.9 : 1. Acute CLE contributed 47.7%, followed by chronic CLE at 46.9% and subacute CLE at 5.4%. A large majority (84%) of patients had systemic lupus erythematosus. Of patients with chronic CLE, about 67.3% developed systemic involvement. Antinuclear antibody (ANA) was detected in 90.0%. Skin biopsy was taken from 42 patients and showed perivascular lymphocytic infiltration (95.2%), epidermal atrophy (47.6%) and hydropic degeneration of the basal layer (47.6%). Immunoglobulin deposition at the dermoepidermal junction was seen in > 40% of patients, predominantly in a granular pattern. Mean CLASI Total was 6.44 ± 7.70, while CLASI Activity (CLASI-A) was 2.75 ± 4.10 and CLASI Damage (CLASI-D) was 3.71 ± 4.76. Involved body surface area (BSA) was found to be an independent predictive factor for CLASI-A (OR = 1.34, P < 0.02). For CLASI-D, positive predictive factors were involved BSA (OR = 4.14, P < 0.001), discoid lupus erythematosus subtype (OR = 13.10, P = 0.001), cutaneous vascular disease (OR = 26.59; P = 0.014), scalp involvement (OR = 8.7, P < 0.01) and hypocomplementaemia (OR = 5.71, P < 0.5). Mean Dermatology Life Quality Index was 5.91 ± 5.34 and correlated significantly with disease severity. CONCLUSIONS: We observed a high percentage of patients with CLE with systemic manifestations and positive ANA result. More aggressive treatment of patients with positive predictive factors for severe disease combined with significant clinical activity may be warranted.
背景：红斑狼疮（CLE）是一种慢性自身免疫性皮肤病，在不同人群中有广泛的临床表现。
目的：研究多民族人群中 CLE 的临床组织学和免疫学特征，并根据皮肤狼疮红斑严重指数（CLASI）确定疾病严重程度的预测因素。
方法：这是一项 2019 年 3 月至 2020 年 2 月进行的 CLE 横断面研究。
结果：共招募了 111 名患者，女性/男性比例为 4.9:1。急性 CLE 占 47.7%，其次是慢性 CLE 占 46.9%，亚急性 CLE 占 5.4%。绝大多数（84%）患者患有系统性红斑狼疮。慢性 CLE 患者中，约 67.3%出现系统受累。90.0%的患者检测到抗核抗体（ANA）。对 42 名患者进行了皮肤活检，显示血管周围淋巴细胞浸润（95.2%）、表皮萎缩（47.6%）和基底层水样变性（47.6%）。免疫球蛋白在表皮真皮交界处沉积>40%的患者中呈颗粒状。平均 CLASI 总分为 6.44±7.70，而 CLASI 活动度（CLASI-A）为 2.75±4.10，CLASI 损伤（CLASI-D）为 3.71±4.76。受累体表面积（BSA）被发现是 CLASI-A 的独立预测因素（OR=1.34，P<0.02）。对于 CLASI-D，阳性预测因素包括受累 BSA（OR=4.14，P<0.001）、盘状红斑狼疮亚型（OR=13.10，P=0.001）、皮肤血管疾病（OR=26.59；P=0.014）、头皮受累（OR=8.7，P<0.01）和低补体血症（OR=5.71，P<0.05）。平均皮肤病生活质量指数为 5.91±5.34，与疾病严重程度显著相关。
结论：我们观察到有相当比例的 CLE 患者有系统表现和 ANA 阳性结果。对于有严重疾病和显著临床活动的患者，结合阳性预测因素，可能需要更积极的治疗。
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