Alexiades-Armenakas Macrene R, Baldassano Marisa, Bince Benji, Werth Victoria, Bystryn Jean-Claude, Kamino Hideko, Soter Nicholas A, Franks Andrew G
New York University School of Medicine, New York, New York 10016, USA.
Arthritis Rheum. 2003 Aug 15;49(4):494-500. doi: 10.1002/art.11206.
To define comprehensive criteria for the classification and differential diagnosis of tumid lupus erythematosus (LE).
A prospective study of patients fulfilling the classical description of tumid LE was performed. Clinical evaluation, histopathologic and direct immunofluorescence analyses of skin specimens, and serologic evaluation were conducted. The inflammatory cell infiltrate was quantitatively investigated by immunohistochemical analysis of fresh frozen skin specimens using multiple lymphocytic markers.
Fifteen patients were followed for a mean of 7 years. Smooth, indurated, nonscarring, pink to violaceous papules, plaques, or nodules, devoid of surface changes were distributed on sun exposed sites. The mean lesion duration was 2 years, female:male ratio was 8:7, and racial distribution was 11 white, 2 Hispanic, and 2 African American patients. Histopathologic findings included a superficial and deep, perivascular, and frequently periadnexal infiltrate of lymphocytes, mucin deposition throughout the dermis, and absent to focal dermal-epidermal junctional involvement. Direct immunofluorescence immunoreactants and low titer antinuclear antibodies were variably present. Immunohistochemical findings included a predominance of pan-T cell marker CD3-expressing (78.0% +/- 6.3%) T lymphocytes. Most were CD4 expressing (82.7% +/- 8.0%) helper T cells; a minority were CD8 expressing (31.3% +/- 14.0%) cytotoxic T cells. The CD4:CD8 ratio was 3.1 (+/-1.3):1. One patient developed systemic LE and one a discoid LE lesion.
Comprehensive clinical, histopathologic, and immunohistochemical criteria for the classification of tumid LE are proposed that differentiate tumid LE from other cutaneous disorders that may be clinically and histologically indistinguishable. The chronic, benign course indicates that tumid LE be classified as a form of chronic cutaneous LE, although it may be a cutaneous feature of systemic LE.
确定肿胀性红斑狼疮（LE）分类及鉴别诊断的综合标准。
对符合肿胀性LE经典描述的患者进行前瞻性研究。进行临床评估、皮肤标本的组织病理学和直接免疫荧光分析以及血清学评估。使用多种淋巴细胞标志物对新鲜冰冻皮肤标本进行免疫组织化学分析，对炎性细胞浸润进行定量研究。
15例患者平均随访7年。光滑、硬结、无瘢痕、粉红色至紫罗兰色丘疹、斑块或结节，无表面改变，分布于暴露部位。平均病损持续时间为2年，女性与男性比例为8:7，种族分布为11名白人、2名西班牙裔和2名非裔美国患者。组织病理学表现包括浅层和深层血管周围以及常为附件周围淋巴细胞浸润、真皮全层粘蛋白沉积以及无至局灶性真皮-表皮交界处受累。直接免疫荧光免疫反应物和低滴度抗核抗体可有可无。免疫组织化学表现包括以表达泛T细胞标志物CD3的（78.0%±6.3%）T淋巴细胞为主。大多数为表达CD4的（82.7%±8.0%）辅助性T细胞；少数为表达CD8的（31.3%±14.0%）细胞毒性T细胞。CD4:CD8比值为3.1（±1.3）:1。1例患者发展为系统性LE，1例出现盘状LE病损。
提出了肿胀性LE分类的综合临床、组织病理学和免疫组织化学标准，以将肿胀性LE与其他在临床和组织学上难以区分的皮肤疾病相鉴别。其慢性、良性病程表明，肿胀性LE应归类为慢性皮肤型LE的一种形式，尽管它可能是系统性LE的一种皮肤表现。
