Zembowicz Artur, Navarro Paula, Walters Stephanie, Lyle Stephen R, Moschella Samuel L, Miller Danielle
Department of Pathology, Lahey Clinic, Burlington, MA 01805, USA.
Am J Dermatopathol. 2011 Dec;33(8):796-802. doi: 10.1097/DAD.0b013e31820edb81.
Ischemic skin necrosis can be a cause of severe morbidity and mortality. It can be due to a number of systemic conditions such as (1) thrombotic vasculopathy syndromes, (2) calciphylaxis, (3) septic or cholesterol emboli, and (4) cutaneous vasculitis. We present 3 patients with a clinicopathological syndrome consisting of ischemic skin necrosis associated with histological pattern of subcutaneous thrombotic vasculopathy-extensive microvascular thrombosis confined to small vessels and capillaries of the subcutaneous tissue. All 3 patients were obese and had severe pre-existing medical conditions. Skin biopsies showed intravascular thrombosis involving small arterioles and capillaries of the subcutaneous tissue. Distribution of vascular involvement by thrombotic process was similar to that observed in calciphylaxis, but calcifications were not observed. Two patients died within 3 months of diagnosis. One patient died 2 years after the presentation. Review of 15 biopsies of calciphylaxis revealed areas of subcutaneous thrombotic vasculopathy in 11 cases (73%). Our study shows that subcutaneous thrombotic vasculopathy syndrome is a potentially lethal condition showing overlapping features between thrombotic vasculopathy syndromes and calciphylaxis. Clinicopathological analysis suggests that it may be a rare variant of calciphylaxis sine calcifications or an early prodromal stage of calciphylaxis. This conclusion is in keeping with increasing appreciation of importance of thrombosis and vascular injury in calciphylaxis.
缺血性皮肤坏死可能导致严重的发病和死亡。它可能由多种全身性疾病引起，如（1）血栓性血管病综合征，（2）钙过敏，（3）脓毒性或胆固醇栓子，以及（4）皮肤血管炎。我们报告了3例患者，他们具有一种临床病理综合征，表现为缺血性皮肤坏死，并伴有皮下血栓性血管病的组织学模式——广泛的微血管血栓形成局限于皮下组织的小血管和毛细血管。所有3例患者均肥胖且有严重的基础疾病。皮肤活检显示皮下组织的小动脉和毛细血管内有血栓形成。血栓形成过程导致的血管受累分布与钙过敏中观察到的相似，但未观察到钙化。2例患者在诊断后3个月内死亡。1例患者在就诊后2年死亡。对15例钙过敏活检的回顾显示，11例（73%）存在皮下血栓性血管病区域。我们的研究表明，皮下血栓性血管病综合征是一种潜在致命的疾病，表现出血栓性血管病综合征和钙过敏之间的重叠特征。临床病理分析表明，它可能是无钙化的钙过敏的一种罕见变体或钙过敏的早期前驱阶段。这一结论与对钙过敏中血栓形成和血管损伤重要性的认识不断增加相一致。
