Overweel Anne F P, Langerak Anton W, Damman Jeffrey, de Haas Ellen L M, Weerkamp Floor, Sandberg Yorick
Erasmus MC, afd. Interne Geneeskunde, Rotterdam.
Erasmus MC, afd. Immunologie, Rotterdam.
Ned Tijdschr Geneeskd. 2022 Feb 16;166:D6288.
Erythroderma could be the first sign of a cutaneous T-cell lymphoma (CTCL), such as Sézary syndrome. Causes of erythroderma include inflammatory dermatosis, toxicoderma, paraneoplastic erytroderma, and CTCL. Hence, diagnosing Sézary syndrome can be difficult. Sézary syndrome is a rare, aggressive disease characterized by erythroderma, generalized lymphadenopathy and the presence of clonally related neoplastic T-cells in skin, peripheral blood, and lymph nodes. Treatment consists of photochemotherapy (PUVA), radiotherapy, immunomodulatory agents, low dose cytotoxic agents, and intensive chemotherapy. Immunotherapy directed against CCR4 and PD1 are new, promising developments.
A 51-year-old man presented with a 1-year history of progressive, itchy erythroderma and lymphocytosis. After extensive cytomorphological, histopathological and molecular examination the diagnosis of Sézary syndrome could be established. Combination treatment of interferon and photochemotherapy (PUVA) was started.
Diagnostic delay in Sézary syndrome is common. Integrated cytomorphological, immunological, and molecular evaluation of peripheral blood in patients with unexplained erythroderma non-responsive to (topical) treatment is warranted.
红皮病可能是皮肤T细胞淋巴瘤（CTCL）的首发症状，如 Sézary 综合征。红皮病的病因包括炎症性皮肤病、中毒性皮肤病、副肿瘤性红皮病和 CTCL。因此，诊断 Sézary 综合征可能具有挑战性。Sézary 综合征是一种罕见的侵袭性疾病，其特征为红皮病、全身性淋巴结肿大以及皮肤、外周血和淋巴结中存在克隆相关的肿瘤性 T 细胞。治疗方法包括光化学疗法（PUVA）、放射疗法、免疫调节剂、低剂量细胞毒性药物和强化化疗。针对 CCR4 和 PD1 的免疫疗法是新的、有前景的进展。
一名 51 岁男性，有 1 年进行性瘙痒性红皮病和淋巴细胞增多病史。经过广泛的细胞形态学、组织病理学和分子检查后，确诊为 Sézary 综合征。开始采用干扰素和光化学疗法（PUVA）联合治疗。
Sézary 综合征的诊断延迟很常见。对于不明原因的对（局部）治疗无反应的红皮病患者，对外周血进行综合的细胞形态学、免疫学和分子评估是必要的。
