Paraneoplastic leukocytoclastic vasculitis mimicking ulcus cruris as rare initial manifestation of smoldering myeloma IgG kappa.

IF 3 3区 医学 Q2 HEMATOLOGY
Annals of Hematology Pub Date : 2025-05-01 Epub Date: 2025-04-21 DOI:10.1007/s00277-025-06366-2
Christina Brummer, Kirsten Utpatel, Sebastian Geis, Sebastian Haferkamp, Andreas Brosig, Markus Herrmann, Chiara Arnreich, Joachim Hahn, Tobias Pukrop, Wolfgang Herr, Sabine Gaerditz
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Abstract

Leukocytoclastic vasculitis (LCV) has been reported as a rare paraneoplastic phenomenon associated with several hematologic disorders, including indolent lymphomas such as Waldenström macroglobulinemia. However, there are very few cases of LCV in the context of plasma cell disorders. We present the case of a 58-year-old female who developed a rapidly progressive, ulceronecrotic skin lesion on her left lower leg due to leukocytoclastic vasculitis. The lesion was initially suspected to be an ulcerative chronic wound (ulcus cruris) but represented an atypical manifestation of leukocytoclastic vasculitis as primary and only clinical sign of smoldering multiple myeloma IgG kappa. After standard induction chemoimmunotherapy with daratumumab, bortezomib, lenalidomide, and dexamethasone, the patient proceeded to high-dose chemotherapy with melphalan, followed by autologous stem cell transplantation for consolidation. Despite a bacterial skin superinfection, myeloma treatment was successfully completed without any major complications. The skin lesion healed concurrently with the reduction in paraprotein levels, and there was no need for plastic surgical intervention. LCV mimicking ulcus cruris can represent a rare and atypical initial manifestation of plasma cell neoplasia. In this case report, systemic myeloma treatment proved to be effective for inducing complete remission of advanced ulceronecrotic skin damage. This case extends the spectrum of reported monocloncal gammopathies of cutaneous significance.

副肿瘤性白细胞破溃性血管炎是阴燃性骨髓瘤IgG kappa罕见的初始表现。
白细胞分裂性血管炎(LCV)是一种罕见的副肿瘤现象,与几种血液系统疾病相关,包括惰性淋巴瘤,如Waldenström巨球蛋白血症。然而,在浆细胞疾病的背景下,很少有LCV病例。我们提出的情况下,58岁的女性谁发展迅速进展,溃疡性坏死皮肤病变在她的左小腿由于白细胞破坏血管炎。该病变最初被怀疑为溃疡性慢性伤口(皱襞),但表现为非典型的白细胞破坏性血管炎,是阴烧性多发性骨髓瘤IgG kappa的主要和唯一临床症状。在使用达拉单抗、硼替佐米、来那度胺和地塞米松进行标准诱导化学免疫治疗后,患者继续使用美法兰进行大剂量化疗,随后进行自体干细胞移植巩固。尽管有细菌性皮肤重复感染,骨髓瘤治疗成功完成,没有任何主要并发症。皮肤损伤愈合的同时,旁蛋白水平降低,不需要整形手术干预。模拟皱褶的LCV可以代表一种罕见和不典型的浆细胞瘤的初始表现。在这个病例报告中,系统性骨髓瘤治疗被证明是有效的诱导完全缓解晚期溃疡坏死皮肤损伤。本病例扩展了已报道的具有皮肤意义的单克隆伽玛病的范围。
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来源期刊
Annals of Hematology
Annals of Hematology 医学-血液学
CiteScore
5.60
自引率
2.90%
发文量
304
审稿时长
2 months
期刊介绍: Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.
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