腿上的紫色病变:除了卡波西肉瘤还有什么?鉴别诊断与文献叙述回顾

A. Pileri, Gionathan Orioni, C. Zengarini, V. Grandi, B. Piraccini, V. Gaspari
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引用次数: 1

摘要

通过这项工作,我们旨在回顾主要影响腿部皮肤的主要良性和恶性肿瘤(包括血管、角化细胞/表皮、黑素细胞、造血和淋巴细胞起源)。在临床实践中,病变的位置也可以帮助集中在一系列的鉴别诊断上。所有的疾病都有相同的临床表现,以红斑到紫色结节为特征。尽管临床表现相同,但每种疾病的预后结果和治疗管理可能有所不同。由于临床诊断有时可能具有挑战性,组织学和免疫组织化学在识别和分期这些类型的病变方面起着重要作用。分子研究可以帮助确定没有特定特征的病变的确切性质。卡波西肉瘤是一种血管增生性肿瘤,通常发生在下肢,可与其他几种罕见的皮肤病进行鉴别诊断。主要的鉴别诊断涉及原发性皮肤淋巴瘤,其中蕈样真菌病是最常见的原发性皮肤t细胞淋巴瘤。其他罕见的形式包括原发性皮肤b细胞淋巴瘤,可分为惰性和侵袭性两种形式,如原发性皮肤弥漫性大b细胞淋巴瘤、腿部型和淋巴瘤样丘疹瘤病(LyP)。在惰性病变的情况下,皮肤定向治疗,有限场放射治疗和手术方法可以是很好的选择。同时,对于侵袭性肿瘤,如母浆细胞样树突状细胞瘤或晚期蕈样真菌病,需要不同的治疗方法,如全身化疗和同种异体骨髓移植。皮肤科医生的角色在识别此类疾病和避免误诊方面至关重要,为病理学家提供准确诊断的正确临床信息,并开始适当的治疗。
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Violaceous Lesions on the Leg: What Else Apart from Kaposi Sarcoma? Differential Diagnosis with a Narrative Review of the Literature
With this work, we aimed to review the principal benign and malignant tumors (including vascular, keratinocytic/epidermal, melanocytic, hematopoietic, and lymphoid origin), primarily affecting the leg’s skin. The lesions’ location can also help focus on a spectrum of differential diagnoses in clinical practice. All the diseases present the same clinical presentation characterized by erythematous to violaceous nodules. Despite the same clinical presentation, each disease’s prognostic outcome and therapeutic management can be somewhat different. Since clinical diagnosis may sometimes be challenging, histology and immunohistochemistry play a fundamental role in recognizing and staging these types of lesions. Molecular studies can help to determine the exact nature of lesions with no specific characteristics. Kaposi’s sarcoma is an angioproliferative neoplasm that typically occurs in the lower limbs and can enter into differential diagnosis with several other rarer skin diseases. The principal differential diagnosis concerns primary cutaneous lymphomas, of which mycosis fungoides represent the most frequent primary cutaneous T-cell lymphoma. Other rare forms include primary cutaneous B-cell lymphomas, which can be divided into indolent and aggressive forms, such as the primary cutaneous diffuse large B-cell lymphoma, leg type, and lymphomatoid papulomatosis (LyP). In the case of indolent lesions, skin-directed therapies, limited-field radiotherapy, and surgical approaches can be good options. At the same time, different management, with systemic chemotherapy and allogenic bone marrow transplant, is required with aggressive neoplasms, such as blastic plasmacytoid dendritic cell neoplasia or advanced mycosis fungoides. The dermatologist’s role can be crucial in recognizing such diseases and avoiding misdiagnosis, giving the pathologist the correct clinical information for an accurate diagnosis, and starting the suitable therapy.
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