PDF(Pubmed)
Abstract：
Sezary syndrome (SS) is a rare but aggressive type of cutaneous T-cell lymphoma (CTCL). Patients with SS have characteristic skin lesions (erythroderma) and a leukemic phase. The rash associated with CTCLs can often mimic common benign skin conditions such as psoriasis, atopic dermatitis, etc. and therefore can go undiagnosed until later stages. We present a case of a patient with SS who managed eczema for over one year with topical steroids before receiving a skin biopsy. Workup confirmed leukemic involvement, and the patient was started on systemic therapy with bexarotene. The patient continues to have a good response to systemic therapy. When treating patients with persistent rash of uncertain etiology and/or unresponsive to treatment, primary care physicians and internists need to consider SS/Mycosis fungoides as a possible differential and should have a low threshold to initiate early referral to dermatology for definitive diagnosis.
摘要：
Sezary综合征（SS）是一种罕见但侵袭性的皮肤T细胞淋巴瘤（CTCL）。SS患者具有特征性皮肤病变（红皮病）和白血病期。与CTCL相关的皮疹通常可以模仿常见的良性皮肤病，如牛皮癣，特应性皮炎,等。因此可能会在后期才被诊断出来。我们介绍了一例SS患者，该患者在接受皮肤活检之前用局部类固醇治疗了一年以上的湿疹。检查证实有白血病参与，患者开始接受贝沙罗汀的全身治疗。患者继续对全身治疗有良好的反应。治疗病因不明和/或对治疗无反应的持续性皮疹患者时，初级保健医师和内科医生需要将SS/真菌菌病作为可能的鉴别因素考虑在内,并且应具有较低的阈值,以便早期转诊至皮肤科进行明确诊断.
