Chan L S, Hanson C A, Cooper K D
Department of Dermatology, University of Michigan Medical School, Ann Arbor.
Arch Dermatol. 1991 Jun;127(6):862-5.
Eosinophilic fasciitis has been reported to precede hematologic malignant neoplasms such as myelomonocytic leukemia, lymphocytic leukemia, and Hodgkin's lymphoma. In this case study, eosinophilic fasciitis occurred concurrently with cutaneous T-cell lymphoma (mycosis fungoides). The clinical diagnosis of eosinophilic fasciitis was based on painful sclerodermatous lesions on the extremities and trunk without acrosclerosis. There was histologic confirmation with edema and lymphocytic inflammation in the superficial muscular fascia and dermis. Deposition of immune reactants was found in the fascia and dermis. In addition, peripheral eosinophilia and circulating immune complexes were detected. The diagnosis of cutaneous T-cell lymphoma (mycosis fungoides) was based on extensive erythematous cutaneous plaques, dermal and epidermal lymphocytic atypia, loss of pan-T-cell immunologic markers, and a cutaneous lesional T-cell receptor beta-chain rearrangement by Southern blot analysis. Eosinophilic fasciitis may occur as a paraneoplastic syndrome associated with hematologic malignant neoplasms, including mycosis fungoides. Cytokines or lymphokines released by activated immunocytes, either malignant leukocytes or normal leukocytes reacting to malignant cells, may be responsible for the eosinophilia and sclerosis seen in these associated hematologic malignant neoplasms.
据报道,嗜酸性筋膜炎可先于血液系统恶性肿瘤出现,如骨髓单核细胞白血病、淋巴细胞白血病和霍奇金淋巴瘤。在本病例研究中,嗜酸性筋膜炎与皮肤T细胞淋巴瘤(蕈样肉芽肿)同时发生。嗜酸性筋膜炎的临床诊断基于四肢和躯干出现疼痛性硬皮病样病变且无肢端硬化。组织学证实浅表肌筋膜和真皮有水肿及淋巴细胞炎症。在筋膜和真皮中发现免疫反应物沉积。此外,检测到外周血嗜酸性粒细胞增多和循环免疫复合物。皮肤T细胞淋巴瘤(蕈样肉芽肿)的诊断基于广泛的红斑性皮肤斑块、真皮和表皮淋巴细胞异型性、泛T细胞免疫标志物缺失以及通过Southern印迹分析发现的皮肤病变T细胞受体β链重排。嗜酸性筋膜炎可能作为一种副肿瘤综合征与血液系统恶性肿瘤相关,包括蕈样肉芽肿。活化的免疫细胞(无论是恶性白细胞还是对恶性细胞起反应的正常白细胞)释放的细胞因子或淋巴因子,可能是这些相关血液系统恶性肿瘤中出现嗜酸性粒细胞增多和硬化的原因。