Pseudoxanthoma Elasticum with Abnormal Nailfold Microcirculatory Findings

彈性纖維假黃瘤是一彈性組織之遺傳疾病。在此，我們報告一34歲男性病患自數月前發現其頸部有許多無自覺症狀黃色小丘疹，當其妹妹告知她也有類似之皮膚症狀與視力受損，即前來本院就診。在皮膚病理切片下經由H&E，Verhoeff-van Gieson以及Von Kossa染色可見在真皮層之中下段有斷裂且鈣化之彈性纖維，在電子顯微鏡下亦可見彈性纖維有鈣化性退化的現象。除了皮膚的病灶與眼底的血管狀條紋外，並無其他器官受影響。此外，經由微血管顯微鏡觀察可見手指甲皺襞處微血管有彎曲程度增加，靜脈端之管徑擴張等變化，同時其血球流速亦有減緩。因此除以往報告中指出因血管壁之彈性纖維退化所引起之心血管疾病表外，本文首度提出彈性纖維假黃瘤患者手指甲皺襞處之微小循環其型態學與血球流速之障礙。

關鍵字

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並列摘要

Pseudoxanthoma elasticum (PXE) is an inherited disorder of elastic tissue. Here we report a 34-year-old male patient who developed multiple symptomless yellowish papules over his neck for several months. He visited our dermatologic out-patient-clinic because his sister had similar skin lesions and mild visual impairment. The pathologic features of the skin biopsy showed fragmented ca1cified elastic fibers in the mid-to-lower dermis under the H&E, Verhoeff-van Gieson and Yon Kossa stains. Under electron microscopy, calcified degenerated elastic fibers were noted. No other internal organ involvement was found except angioid streaks on the fundus. In addition, morphological changes of the nailfold capillaries, including increased tortuosity, dilated venous limbs of capillary loops, and decreased red-blood-cell velocity, were observed under tbe capillaroscopy. Though former reports have indicated that cardiovascular manifestations are caused by degeneration of elastic fibers of blood vessels, this study is the first to emphasize the microcirculatory disturbance of nail fold capillary, including morphology and blood-cell velocity, in PXE.

並列關鍵字

pseudoxanthoma elasticum； elastic fiber； microcirculation

國際替代計量

Pseudoxanthoma Elasticum with Abnormal Nailfold Microcirculatory Findings

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