Pseudolymphomatous Cutaneous Angiosarcoma Presenting With Persistent Firm Facial Edema in a Patient With Systemic Sclerosis.

IF 1.1 4区 医学 Q4 DERMATOLOGY American Journal of Dermatopathology Pub Date : 2024-09-01 Epub Date: 2024-06-06 DOI:10.1097/DAD.0000000000002740
Silvia Méndez-Flores, Marcela Saeb-Lima, Hilda Esther Fragoso-Loyo
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Abstract

Abstract: Pseudolymphomatous cutaneous angiosarcoma (cAS) is a rare subtype characterized by a prominent lymphocytic infiltrate, posing diagnostic challenges due to its resemblance to lymphoid neoplastic processes. We present a novel case highlighting the clinical and histopathological features, notably its association with persistent firm facial edema in a patient with systemic sclerosis (SSc). A 47-year-old woman with a 21-year history of SSc presented with firm palpebral edema evolving to involve the entire face and cervical region over six months. Diagnostic imaging revealed inflammatory changes in orbital regions, supradiaphragmatic lymphadenopathies, and lytic lesions. Skin biopsy demonstrated a diffuse neoplasm with vascular channels and solid areas, accompanied by dense lymphocytic proliferation. Pseudolymphomatous cutaneous angiosarcoma, a rare malignant neoplasm, exhibits variable clinical presentations and rapid progression. Histologically, it manifests as irregularly shaped vascular channels lined by prominent endothelial cells. Immunohistochemistry, particularly markers such as v-ets erythroblastosis virus E26 oncogene homolog (avian) (ERG), aids in diagnosis. Notably, this case marks the first presentation of cAS with persistent facial edema in SSc, highlighting the association between SSc and cancer risk. This case underscores the diagnostic challenges posed by cAS and emphasizes the importance of early detection for optimal patient outcomes. Further understanding of its association with autoimmune disorders such as SSc is crucial for comprehensive management strategies.

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假性淋巴瘤性皮肤血管肉瘤伴有系统性硬化症患者面部持续性坚实水肿
摘要:假性淋巴瘤性皮肤血管肉瘤(cAS)是一种罕见的亚型,其特点是淋巴细胞浸润明显,由于与淋巴肿瘤过程相似,给诊断带来了挑战。我们介绍了一个新病例,该病例强调了其临床和组织病理学特征,尤其是它与一名系统性硬化症(SSc)患者面部持续性坚实水肿的关联。一名 47 岁的女性患者有 21 年的系统性硬化症病史,半年来出现睑结膜水肿,并逐渐累及整个面部和颈部。诊断性影像学检查发现眼眶部位有炎症性改变、膈上淋巴结病变和溶解性病变。皮肤活检显示肿瘤呈弥漫性,有血管通道和实变区,伴有密集的淋巴细胞增生。假性淋巴瘤性皮肤血管肉瘤是一种罕见的恶性肿瘤,临床表现各异,病情发展迅速。组织学上,它表现为由突出的内皮细胞衬托的形状不规则的血管通道。免疫组化,尤其是 v-ets 红细胞增多症病毒 E26 癌基因同源物(禽)(ERG)等标记物有助于诊断。值得注意的是,该病例是首次在 SSc 患者中出现伴有持续性面部水肿的 cAS 病例,突显了 SSc 与癌症风险之间的关联。该病例凸显了 cAS 在诊断方面的挑战,并强调了早期发现对患者获得最佳治疗效果的重要性。进一步了解它与 SSc 等自身免疫性疾病的关联对于制定全面的管理策略至关重要。
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来源期刊
CiteScore
1.80
自引率
9.10%
发文量
453
审稿时长
3 months
期刊介绍: The American Journal of Dermatopathology offers outstanding coverage of the latest diagnostic approaches and laboratory techniques, as well as insights into contemporary social, legal, and ethical concerns. Each issue features review articles on clinical, technical, and basic science advances and illuminating, detailed case reports. With the The American Journal of Dermatopathology you''ll be able to: -Incorporate step-by-step coverage of new or difficult-to-diagnose conditions from their earliest histopathologic signs to confirmatory immunohistochemical and molecular studies. -Apply the latest basic science findings and clinical approaches to your work right away. -Tap into the skills and expertise of your peers and colleagues the world over peer-reviewed original articles, "Extraordinary cases reports", coverage of practical guidelines, and graphic presentations. -Expand your horizons through the Journal''s idea-generating forum for debating controversial issues and learning from preeminent researchers and clinicians
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