Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis
Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis
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DOI：
10.1001/archderm.140.5.591
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发表时间：
2004-05-01
影响因子：
--
通讯作者：
Frieden, IJ
中科院分区：
文献类型：
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作者：
Metry, DW;Hawrot, A;Frieden, IJ
背景：皮肤多发性血管瘤传统上被认为是潜在的内脏血管瘤的线索。最近，血管瘤被认为有亚型、局限型和节段型，与并发症的风险相关。虽然不太常见，但皮肤节段性血管瘤威胁生命或功能和/或有相关结构异常的风险较高，例如发生在Phace综合征(后颅窝脑畸形、血管瘤、动脉异常、主动脉缩窄和心脏缺陷以及眼睛异常)综合征(PHACES，如果包括胸骨裂/脐上缝)。然而，孤立的节段性皮肤血管瘤与内脏血管瘤病的潜在联系以前并没有被强调。观察：共有47例皮肤节段性血管瘤与内脏血管瘤病相关。皮肤血管瘤的部位最常见，但并非仅限于面部(37例[79%])。最常见的内脏受累部位是肝脏(20例(43%))，其次是胃肠道(16例[34%])、脑(16例[34%])、纵隔(9例[19%])和肺(7例[15%])。报告的胰腺、脾、骨或肾脏血管瘤的百分比为6%或更低。40%的患者符合相思(S)综合征的诊断标准。在这一亚组中，内脏血管瘤最常见于脑或纵隔(18例[53%])。总体而言，12名患者(25%)在婴儿期死亡，最常见的原因是胃肠道受累或继发于肝脏受累的充血性心力衰竭。结论：皮肤节段性血管瘤与内脏血管瘤病相关，并有可能导致重要器官受损。
Background: Multiple hemangiomas of the skin have traditionally been recognized as a clue to potential visceral hemangiomas. Recently, hemangiomas have been recognized to have subcategories, localized and segmental, which correlate with risk of complications. While less common, segmental hemangiomas of the skin have a higher risk of being life- or function-threatening and/or having associated structural anomalies such as those that occur in PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) syndrome (PHACES, if sternal clefting/supraumbilical raphe is included). However, the potential association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis has not been previously emphasized.Observations: A total of 47 cases of segmental hemangiomas of the skin in association with visceral hemangiomatosis were found. The location of the cutaneous hemangiomas most commonly, but not exclusively, involved the face (37 cases [79%]). The most common site of internal organ involvement was the liver (20 cases [43%]), followed by the gastrointestinal tract (16 [34%]), brain (16 [34%]), mediastinum (9 [19%]), and lung (7 [15%]). The percentages of reported cases of hemangiomas of the pancreas, spleen, bones, or kidneys were 6% or less. Forty percent of patients met criteria for the diagnosis of PHACE(S) syndrome. In this subgroup, internal organ hemangiomas were most commonly found in the brain or mediastinum (18 cases [53%]). Overall, 12 patients (25%) died during infancy, most commonly because of gastrointestinal involvement or congestive heart failure secondary to liver involvement.Conclusion: Segmental hemangiomas of the skin have an associated risk of visceral hemangiomatosis, with the potential of causing vital organ compromise.