Tagami Travis, Alhalabi Omar, Ward Nicholas, Huang James
Department of Internal Medicine, William Beaumont Hospital, Oakland University William Beaumont School Medicine, Royal Oak, Mich., USA.
Department of Pathology, William Beaumont Hospital, Oakland University William Beaumont School Medicine, Royal Oak, Mich., USA.
Dermatopathology (Basel). 2016 Apr 30;3(2):39-43. doi: 10.1159/000445766. eCollection 2016 Apr-Jun.
BACKGROUND/AIMS: Paraneoplastic dermatoses are skin disorders that are associated with malignancy. Anaplastic large T-cell lymphoma (ALTCL) has rarely been associated with paraneoplastic skin manifestations such as gangrenous foot ulcers and erythroderma.
We describe a case of ALTCL presenting as a large annular skin rash. The clinical picture, course, and treatment will be discussed along with current hypotheses on the mechanism of paraneoplastic syndromes.
Skin manifestations in ALTCL most commonly arise in two distinct ways; either as primary cutaneous lymphoma manifestation or as systemic disease with secondary metastasis. Less commonly, systemic disease causes skin manifestations secondary to a paraneoplastic process without infiltration of malignant cells. This is thought to be mediated by an immunologic reaction to tumor antigen or the result of cytokines and other inflammatory markers produced by the tumor itself.
Paraneoplastic dermatoses could be the initial presentations of systemic lymphoma. Knowledge about their association with anaplastic large-cell lymphoma may help with timely diagnosis. In a patient with unexplained dermatosis associated with B symptoms who is unresponsive to topic treatment, an investigation for systemic lymphoma workup is warranted.
背景/目的:副肿瘤性皮肤病是与恶性肿瘤相关的皮肤疾病。间变性大T细胞淋巴瘤(ALTCL)很少与坏疽性足部溃疡和红皮病等副肿瘤性皮肤表现相关。
我们描述了1例表现为巨大环形皮疹的ALTCL病例。将结合目前关于副肿瘤综合征机制的假说讨论临床表现、病程及治疗情况。
ALTCL的皮肤表现最常见于两种不同方式;要么作为原发性皮肤淋巴瘤表现,要么作为伴有继发性转移的全身性疾病。较少见的是,全身性疾病导致副肿瘤性过程继发皮肤表现,而无恶性细胞浸润。这被认为是由对肿瘤抗原的免疫反应介导的,或是肿瘤本身产生的细胞因子和其他炎症标志物的结果。
副肿瘤性皮肤病可能是全身性淋巴瘤的初始表现。了解它们与间变性大细胞淋巴瘤的关联可能有助于及时诊断。对于患有无法解释的皮肤病且伴有B症状且对局部治疗无反应的患者,有必要进行全身性淋巴瘤检查。