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首页> 外文期刊>Journal of cutaneous pathology >Pseudoxanthoma elasticum-like fibers in the inflamed skin of patients without pseudoxanthoma elasticum.
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Pseudoxanthoma elasticum-like fibers in the inflamed skin of patients without pseudoxanthoma elasticum.

机译：无弹性假黄瘤的患者发炎的皮肤中出现假弹性黄瘤样纤维。

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Background: Pseudoxanthoma elasticum (PXE) is an inherited disorder leading to characteristic calcified elastic fibers in skin, eyes and vasculature. PXE-like fibers have not been described in inflammatory skin disease in the absence of other signs of PXE. Methods: The histopathology of inflamed skin from 13 patients that contained PXE-like fibers but lacked clinical evidence of PXE were studied. Six of these and six comparison specimens from known patients with PXE were subjected to polymerase chain reaction amplification and sequencing of exons 24 and 28 of the PXE-associated gene ABCC6. This genetic analysis employed a novel assay utilizing paraffin-embedded tissue. Results: Incidental PXE-like fibers were found in patients without clinical suspicion of PXE in lesional tissue showing lipodermatosclerosis, granuloma annulare, lichen sclerosus, morphea profunda, erythema nodosum, septal panniculitis, basal cell carcinoma and fibrosing dermatitis. Two patients with PXE-like fibers but without clinical findings of PXE were heterozygous for a PXE-associated ABCC6 sequence alteration. Conclusions: This pilot study shows elastic fibers similar to those of PXE in the lesional skin of patients with a variety of inflammatory skin diseases in the absence of clinical evidence of PXE; and some of these patients harbor changes in ABCC6.
机译：背景：弹性假黄瘤（PXE）是一种遗传性疾病，导致皮肤，眼睛和脉管系统中钙化的弹性纤维具有特征性。在没有其他PXE征象的情况下，尚未在炎症性皮肤病中描述过PXE样纤维。方法：对13例含有PXE样纤维但缺乏PXE临床证据的皮肤发炎的组织病理学进行了研究。将来自已知患有PXE的患者中的六个和六个比较标本进行聚合酶链反应扩增，并对PXE相关基因ABCC6的外显子24和28进行测序。该遗传分析采用了一种利用石蜡包埋的组织的新颖测定方法。结果：在没有临床怀疑PXE的患者的病变组织中发现了偶然的PXE样纤维，表现为脂肪性皮硬化，环形肉芽肿，地衣性硬化，原发性吗啡，结节性红斑，中隔性脂膜炎，基底细胞癌和纤维化性皮炎。两名患有PXE样纤维但无PXE临床表现的患者因PXE相关的ABCC6序列改变而杂合。结论：该初步研究显示，在缺乏PXE临床证据的情况下，患有多种炎性皮肤病的患者的病变皮肤中的弹性纤维与PXE相似;其中一些患者的ABCC6发生改变。

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