Mayo Clinic, Scottsdale, Arizona.
Mayo Clinic, Rochester, Minnesota; New York University, New York, New York.
J Am Acad Dermatol. 2017 Oct;77(4):719-727. doi: 10.1016/j.jaad.2017.05.036. Epub 2017 Jul 11.
Classic Sézary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease.
To describe the early clinical characteristics of patients with SS.
A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015.
Erythroderma was the earliest recorded skin sign of SS in only 25.5% of cases, although most patients (86.3%) eventually developed erythroderma. In patients without erythroderma during their initial visit, the first cutaneous signs of SS were nonspecific dermatitis (49%), atopic dermatitis-like eruption (4.9%), or patches and plaques of mycosis fungoides (10.6%). The mean diagnostic delay was 4.2 years overall, 2.2 years for cases involving erythroderma at the initial presentation, and 5.0 years for cases not involving erythroderma at the initial presentation.
This study is retrospective.
Erythroderma is uncommon as an initial sign of SS. Early SS should be considered in cases of nonerythrodermic dermatitis that is refractory to conventional treatments. In these cases, examination of the blood by PCR for monoclonal T-cell receptor rearrangement and by flow cytometry to identify an expanded or aberrant T-cell population should be considered.
经典 Sézary 综合征（SS）的定义为红皮病、全身性淋巴结病和白血病血受累。临床观察表明，SS 始于非红皮病。
描述 SS 患者的早期临床特征。
对 1976-2015 年期间确诊的 263 例 SS 患者进行回顾性、多中心病历回顾。
尽管大多数患者（86.3%）最终出现红皮病，但仅有 25.5%的病例最早记录的 SS 皮肤表现为红皮病。在初次就诊时无红皮病的患者中，SS 的最初皮肤表现为非特异性皮炎（49%）、特应性皮炎样疹（4.9%）或蕈样真菌病斑块和斑块（10.6%）。总的来说，平均诊断延迟为 4.2 年，初次就诊时出现红皮病的病例为 2.2 年，初次就诊时无红皮病的病例为 5.0 年。
本研究为回顾性。
红皮病作为 SS 的初始表现并不常见。对于常规治疗无效的非红皮病性皮炎，应考虑早期 SS。在这些情况下，应考虑通过 PCR 检测单克隆 T 细胞受体重排和流式细胞术检查血液以识别扩展或异常 T 细胞群体，以确诊 SS。
