Dysregulated Expression of Antimicrobial Peptides in Skin Lesions of Patients with Cutaneous T-cell Lymphoma.
- 作者列表："Wehkamp U","Jost M","Wehkamp K","Harder J
:Mycosis fungoides and Sézary syndrome belong to the group of primary cutaneous T-cell lymphomas. Because of the inflammatory appearance of the skin lesions, we hypothesized that antimicrobial peptides might be dysregulated in these conditions, similar to in inflammatory skin conditions. Samples from 20 patients with cutaneous T-cell lymphoma were analysed using immunohistochemistry and enzyme-linked immunoassay (ELISA) of skin washing fluids of hBD-2, hBD-3, RNase 7 and psoriasin. Immunohistochemistry results were compared with previous analyses of healthy and psoriatic skin. ELISA and immunohistochemistry revealed a higher expression of psoriasin in lesional cutaneous T-cell lymphoma compared with non-lesional and healthy samples. Immunohistochemistry showed an increase in hBD-2 in lesional cutaneous T-cell lymphoma skin compared with healthy skin. The expression profile of antimicrobial peptides in cutaneous T-cell lymphoma appears to be dysregulated, indicating a potential role of antimicrobial peptides in cutaneous T-cell lymphoma. A larger prospective study and functional studies are needed to improve our understanding of the role of antimicrobial peptides in cutaneous T-cell lymphoma.
: 蕈样肉芽肿和s é zary综合征属于原发性皮肤T细胞淋巴瘤。由于皮损的炎症表现，我们假设抗菌肽在这些条件下可能失调，类似于炎症性皮肤条件。对 20 例皮肤T细胞淋巴瘤患者的皮肤冲洗液进行免疫组织化学和酶联免疫分析 (ELISA)，检测hBD-2 、hBD-3 、RNase 7 和银屑病皮损。将免疫组织化学结果与以前对健康和银屑病皮肤的分析进行比较。ELISA和免疫组化显示银屑病在皮损型皮肤T细胞淋巴瘤中的表达高于非皮损型和健康对照。免疫组化显示与健康皮肤相比，皮损皮肤T细胞淋巴瘤皮肤的hBD-2 增加。皮肤T细胞淋巴瘤中抗菌肽的表达谱似乎失调，表明抗菌肽在皮肤T细胞淋巴瘤中的潜在作用。需要更大的前瞻性研究和功能研究来提高我们对抗菌肽在皮肤T细胞淋巴瘤中的作用的理解。
METHODS::Blue rubber bleb naevus syndrome (BRBNS) is an extremely rare venous malformation that often manifests as multiple haemangioma-like lesions in the skin and gastrointestinal tract. The drug sirolimus plays a key role in the signalling pathway of angiogenesis and subsequent development of BRBNS and its use has been described in several case reports. We present a case series of four patients with BRBNS who exhibited good treatment response to sirolimus. All four patients were administered oral sirolimus at doses of 1.0-1.5 mg/m2 /day with a target drug level of 5-10 ng/mL and median treatment duration of 20 months. All patients had a reduction in the size of the lesions and a normalization of coagulopathy with tolerable drug adverse reactions at follow-up. Sirolimus may be effective and safe in paediatric patients with BRBNS. Further prospective studies are suggested to evaluate the long-term effectiveness of this drug.
METHODS:BACKGROUND:Human papillomavirus (HPV) infections are associated with common dermatologic and nondermatologic diseases. Although HPV vaccines are well established as preventive measures for genital warts and cervical neoplasia, their use as therapeutic agents deserves greater attention. OBJECTIVE:To evaluate the use of HPV vaccine(s) as a treatment modality for cutaneous and/or mucosal disease. METHODS:A primary literature search using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted in January 2019 by using the PubMed and Cochrane databases. RESULTS:A total of 63 articles with 4439 patients were included. The majority of patients with cutaneous warts, recurrent respiratory papillomatosis, and squamous and basal cell carcinomas were successfully treated with HPV vaccination. Preliminary data on patients with pre-existing anogenital warts, cervical intraepithelial neoplasia, anal intraepithelial neoplasia, and vulvar intraepithelial neoplasia is promising. LIMITATIONS:This review was limited by the lack of controls, patients' previous HPV vaccination status, and publication bias. CONCLUSION:The commercially available three-dose, quadrivalent HPV vaccine is a potential therapeutic option for the treatment of cutaneous warts, recurrent respiratory papillomatosis, and squamous and basal cell carcinomas. Noncommercially available HPV vaccines demonstrate therapeutic response for treating anogenital warts, cervical intraepithelial neoplasia, anal intraepithelial neoplasia, and vulvar intraepithelial neoplasia. The vaccine's efficacy as an adjunct therapy for HPV-associated cutaneous and/or mucosal disease warrants further exploration.
METHODS::Our understanding of melanoma precursors and progression to melanoma has developed as a result of advances in the field of molecular diagnostics. We now better understand the potential for genetic heterogeneity within a single lesion. Combined tumors can pose a diagnostic challenge when deciding the line between benign and malignant, which in turn has direct implications for patient management. Primary cilia (PC) are ubiquitous sensory organelles that have essential functions in cellular proliferation, differentiation, and development. The ciliation index (percentage of ciliated melanocytes) has been shown to reliably differentiate melanoma, which fail to ciliate, from melanocytic nevi, which retain PC. We therefore analyzed the potential for using the ciliation index to differentiate benign and malignant components in combined melanocytic lesions. We collected patient samples (n = 10) of unequivocal combined lesions with both melanoma and associated nevus components. Melanocytes were highlighted with SOX10 and costained with gamma-Tubulin and acetylated alpha-Tubulin to highlight the basal body and cilium, respectively. The number of melanocytes retaining cilia under high-power microscopy was examined. The melanoma component had average of 4% ciliation (SD: 7%), whereas the associated nevus component was significantly higher with 59% ciliation (SD: 17%). These data show that PC may be a reliable means of distinguishing benign from malignant components within a single tumor. The ciliation index may be a helpful tool in distinguishing challenging cases of combined lesions of melanoma in situ with a dermal nevus component from invasive melanoma, thus promoting improved staging and clinical management.