副肿瘤性庞贝氏症:临床和诊断特征 作者

A. Karamova, Ludmila F. Znamenskaya, M. Nefedova, Egana S. Mekhdieva, Ekaterina V. Ekaterina
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摘要

副肿瘤性丘疹性荨麻疹是一种以肿瘤为背景的自身免疫性皮肤病。它可以发生在癌症确诊之前、治疗期间或治疗后不久,甚至在潜在疾病缓解期间。通常情况下,副肿瘤性丘疹性荨麻疹仅指 IgG 的沉积。本文主要讨论的是副肿瘤性 IgA/IgG 丘疹性荨麻疹,它是副肿瘤性丘疹性荨麻疹的一种罕见形式,在这种情况下,IgA 和 IgG 同时沉积。我们描述了一例在慢性淋巴细胞白血病缓解期出现各种皮疹的患者的临床病例。副肿瘤性 IgA/IgG 丘疹病的诊断基于临床和病史资料、诊断性皮肤活检结果、免疫荧光试验、血液科医生会诊和实验室检查。诊断副肿瘤性丘疹病的困难在于皮疹的多态性以及临床和实验室参数的多样性,这可能取决于并发的肿瘤病理。诊断需要对疾病的临床表现进行评估,仔细收集病史资料,并为大疱性皮肤病患者制定一套完整的诊断算法。
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PARANEOPLASTIC PEMBIGUS: CLINICAL AND DIAGNOSTIC FEATURES AUTHORS
Paraneoplastic pemphigus is an autoimmune dermatosis following against the background of neoplasia. It can occur before cancer is diagnosed, during or shortly after treatment, and even during remission of the underlying disease. Typically, paraneoplastic pemphigus only refers to the deposition of IgG. The article is devoted to paraneoplastic IgA/IgG pemphigus, which is one of the rare forms of paraneoplastic pemphigus in which deposition of IgA together with IgG is observed. We have described a clinical case of a patient who developed various rashes during the period of remission of chronic lymphocytic leukemia. Diagnosis of paraneoplastic IgA/IgG pemphigus based on clinical and anamnestic data, the results of a diagnostic skin biopsy, immunofluorescence test, consultation with a hematologist and lab tests. Difficulties in diagnosing paraneoplastic pemphigus are due to the polymorphism of rashes and the variety of clinical and laboratory parameters, which may depend on concomitant oncological pathology. A diagnostic search requires an assessment of the clinical picture of the disease, a carefully collected anamnesis, and a complete diagnostic algorithm for patients with bullous dermatoses
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