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红皮病:银屑病还是淋巴瘤?诊断挑战和治疗陷阱。

Erythroderma: psoriasis or lymphoma? A diagnostic challenge and therapeutic pitfall.

机构信息

Division of Dermatology, University Hospital of Bologna, Bologna, Italy.

Division of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

出版信息

Ital J Dermatol Venerol. 2022 Apr;157(2):154-157. doi: 10.23736/S2784-8671.21.06891-7. Epub 2021 Apr 29.

Abstract

BACKGROUND

Psoriasis and lymphoma risk is widely debated, but few is known about misdiagnosis risk between erythrodermic psoriasis and lymphoma. In fact, erythroderma might represent a clinical presentation of psoriasis, cutaneous T-cell lymphomas and skin dissemination of systemic lymphomas.

METHODS

All patients referred to psoriasis outpatient service with a diagnosis of erythrodermic psoriasis were re-examined. Among them, all the patients with a subsequent lymphoma diagnosis were included. For each patient data concerning age, gender, age at erythroderma onset, age at lymphoma diagnosis, immune-suppressive therapy, type of lymphoma and relative stage, lymphoma treatment and outcome were obtained.

RESULTS

Twenty-five patients (15 females and 10 males) with a diagnosis of erythrodermic psoriasis were retrieved. Among them, 9 patients (5 males and 4 females) were affected by erythrodermic lymphoma, including 4 patients with Sèzary Syndrome, 3 with mycosis fungoides, and 2 with peripheral T-cell lymphoma not otherwise specified. Prior to lymphoma diagnosis all the patients (9/9) received cyclosporine, two (2/9) of them methotrexate, one (1/9) azatioprine, and two (2/9) systemic corticosteroids. The prognosis of our patients was poor, due to immune-suppressive drugs administration in patients with undiagnosed lymphoma. The only exception was one (1/9) patient with Sèzary Syndrome still alive with disease after 120 months of follow-up.

CONCLUSIONS

In case of patients with erythroderma, multiple skin biopsies and specific peripheral blood studies like flow cytometry and T-cell receptor gene rearrangement analysis are required in order to avoid misdiagnosis risk between psoriasis and lymphoma.

摘要

背景

银屑病和淋巴瘤的风险备受争议,但人们对红皮病型银屑病和淋巴瘤之间的误诊风险知之甚少。事实上,红皮病可能代表银屑病、皮肤 T 细胞淋巴瘤和全身性淋巴瘤皮肤播散的一种临床表现。

方法

所有因红皮病型银屑病而就诊于银屑病门诊服务的患者均接受了重新检查。其中,所有随后被诊断为淋巴瘤的患者均被纳入研究。对于每一位患者,我们均获取了与年龄、性别、红皮病发病年龄、淋巴瘤发病年龄、免疫抑制治疗、淋巴瘤类型和相对分期、淋巴瘤治疗和结局相关的数据。

结果

共检索到 25 例(15 名女性和 10 名男性)红皮病型银屑病患者。其中,9 例(5 名男性和 4 名女性)患有红皮病型淋巴瘤,包括 4 例蕈样肉芽肿,3 例皮肤 T 细胞淋巴瘤,2 例未特指的外周 T 细胞淋巴瘤。在诊断为淋巴瘤之前,所有患者(9/9)均接受了环孢素治疗,其中 2 例(2/9)还接受了甲氨蝶呤治疗,1 例(1/9)接受了硫唑嘌呤治疗,2 例(2/9)接受了全身皮质类固醇治疗。由于在未确诊的淋巴瘤患者中使用了免疫抑制剂,我们患者的预后较差。唯一的例外是 1 例(1/9)患有蕈样肉芽肿的患者在随访 120 个月后仍有疾病存在但仍存活。

结论

对于红皮病患者,需要进行多次皮肤活检和特定的外周血检查,如流式细胞术和 T 细胞受体基因重排分析,以避免银屑病和淋巴瘤之间的误诊风险。

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