Hidden Heart Disease: 19 Dermatologic Clues You Should Know
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | June 13, 2017
心脏病患者常常有皮肤表现。例如，上图显示一名结节病患者（结节病是一种多系统炎性疾病）的冻疮样狼疮，表现为鼻尖部和鼻翼的结节。这种皮肤表现对于指导临床医生作出心脏病的正确诊断起到非常重要的作用。一些皮肤表现一旦出现，提醒临床医生应当进一步检查心血管系统。本幻灯旨在检验你对于这些皮肤表现的了解程度。
皮肤表现是胆固醇栓塞的最常见临床表现；根据一项系统回顾，大约34%的患者具有皮肤表现。图中所示为足底的网状青斑，是胆固醇栓塞最常见的皮肤表现，约16%的患者可以发生。网状青斑的特点是皮肤红色花斑，加压后褪色。超过1/4的病例出现脚趾疼痛紫绀。
Image from Medscape.
问题1：以下有关胆固醇栓塞诊断的说法哪个是正确的？
答案1：A. 已知罹患动脉粥样硬化的患者若有典型的肾衰、腹痛及网状青斑病史，应高度怀疑胆固醇栓塞
Although the most frequent triggering event is an invasive vascular procedure that dislodges an unstable plaque, cases of spontaneous cholesterol embolism have been reported. Some cases directly follow the triggering event, while others are delayed by months. [2] Findings of Hollenhorst plaques of the retina have been seen following arteriography, cardiac catheterization, vascular surgery, or trauma to the abdomen. Pedal pulses are present in over 60% of patients with cholesterol embolism. [3] Laboratory testing is usually nonspecific and may show an elevated white blood cell count, a decreased red blood cell count, and elevated inflammatory markers, such as C-reactive protein, erythrocyte sedimentation rate, and fibrinogen. [4]
尽管最常见的诱因是有创血管操作导致斑块不稳定，但也有自发性胆固醇栓塞的病例报告。部分病例在诱因出现后立即发病，而其他病例的发病延迟数月。视网膜Hollenhorst斑块可见于动脉造影、心脏导管、血管外科手术或腹部创伤。超过60%的胆固醇栓塞足背动脉脉搏存在。实验室检查缺乏特异性，可见白细胞计数升高，红细胞计数减少，炎症介质如C反应蛋白、血沉及纤维蛋白原升高。
The above image also represents a cutaneous manifestation of cholesterol embolism. It is from a 76-year-old man with a history of aorto-bifemoral bypass graft who developed this eruption after an angiographic procedure. The plantar surface of the right foot shows discoloration resulting from petechiae arranged in a reticulated pattern. This is not livedo reticularis. (Unlike the lesions of livedo reticularis, petechiae do not blanch on diascopy.)
上图显示胆固醇栓塞的皮肤表现。这是一名76岁男性，既往史包括主动脉双侧股动脉人工血管搭桥术。患者在一次血管造影后出现如图表现。右足足底淤点呈网状分布。这并非网状青斑。（与网状青斑不同，加压后淤点不褪色）
Image from Medscape.
A skin biopsy may be helpful in diagnosing cholesterol embolism. This image shows an arteriole occluded with a thrombus laden with needle-shaped cholesterol clefts (yellow arrow). Classically, histology demonstrates needle-shaped cholesterol clefts and intravascular microthrombi. (However, despite these findings being specific for a cholesterol embolus, histology may not correlate with the clinical picture.) The site of biopsy should be directly over the site of the suspected embolus, but caution must be taken or biopsy avoided if obvious ischemia is present. Histologic diagnosis is made by observation of positive birefringent crystals with plane polarized light or by visualizing biconvex clefts within an arterial lumen. [4]
皮肤活检有助于诊断胆固醇栓塞。上图显示小动脉被内含针尖样胆固醇结晶的血栓阻塞（黄色箭头）。典型情况下，组织学检查显示针尖样胆固醇结晶及血管内微血栓。（然而，尽管上述发现对于诊断胆固醇栓塞具有特异性，但组织学结果可能与临床表现无关。）应当在可疑栓塞的部位进行活检，但若存在缺血，则应格外谨慎或避免进行活检。如果观察到双折射晶体及平面偏振光，或看到动脉管腔内双凸形结晶，则可以做出组织学诊断。
Image from Medscape.
Sarcoidosis—a multisystem inflammatory disease of unknown etiology characterized by non-caseating granulomas occurring in organs and tissue—manifests itself on the skin in 25% of patients. Plaque sarcoidosis (shown) features lesions that are round to oval and red-brown to purple and are most commonly located on the extremities, face/scalp, back, and buttocks; the distribution is usually symmetrical.
结节病是一种多系统炎性疾病，病因未明，特点是器官和组织非干酪样肉芽肿。25%的患者具有皮肤表现。斑块样结节病（图示）的特点为圆形至椭圆形、棕红色至紫色病变，最常见于肢体、面部/头皮、背部和臀部；病变通常呈对称性分布。
Although the most common organ involved in sarcoidosis is the lung, cardiac involvement may occur in up to 30% of patients, [5] with intrathoracic lymph nodes also being frequently involved. Plaque sarcoidosis generally has a chronic cutaneous manifestation (>2 years) associated with more severe systemic involvement. [6]
结节病最常受累的器官为肺，但多达30%的患者可有心脏受累，胸腔内淋巴结也经常受累。斑块样结节病的皮肤表现通常为慢性（> 2年），且伴有非常严重的全身受累。
In clinically evident cardiac sarcoidosis, the most common finding is complete heart block, which occurs at a younger age than it does when arising from another cause. [5,7]
在临床表现明显的心脏结节病，最常见的表现为完全性心脏传导阻滞，较其他疾病引起的心脏传导阻滞发病年龄更轻。
Image from Wikimedia Commons | M Sand, D Sand, C Thrandorf, V Paech, P Altmeyer, FG Bechara.
The patient in the above image has lupus pernio, a cutaneous sign of sarcoidosis. Lupus pernio is an indolent and often disfiguring red to purple nodular or plaque-like, sarcoidotic skin lesion that may affect the cheeks, nose, chin, forehead, or ears or the perioral or periocular regions. The presence of lupus pernio is associated with an elevated risk for extracutaneous disease, especially pulmonary involvement. [8]
上图患者为冻疮样狼疮，为结节病的皮肤表现。冻疮样狼疮呈缓慢进展，常常表现为红色至紫色结节或斑块样结节病样皮损，可累及颊部、鼻、颏、前额、耳、口周或眶周区域。冻疮样狼疮常伴随皮肤外表现风险增加，尤其是肺部受累。
Image from Medscape.
The most frequent nonspecific cutaneous manifestation of sarcoidosis is erythema nodosum (shown). Erythema nodosum can be present in 20% of all patients with sarcoidosis and up to 62% of patients with cutaneous manifestations.
结节病最常见的非特异性皮肤表现为结节性红斑（图示）。结节性红斑可见于20%的结节病患者，多达62%的有皮肤表现的结节病患者具有结节性红斑。
Image from Medscape.
问题2：对于可疑结节病患者的鉴别诊断应当包括以下哪项？
答案2：E. 以上所有
A skin biopsy of plaque sarcoidosis or lupus pernio would likely reveal noncaseating granuloma (shown), although a biopsy of erythema nodosum would not. Chest radiography is warranted because the most common organs of involvement in sarcoidosis are the lungs and intrathoracic lymph nodes; such imaging usually reveals bilateral hilar lymph node enlargement with normal lungs. A 24-hour Holter monitor is indicated because sudden death, caused by ventricular tachyarrhythmias or bradyarrhythmias, can result from cardiac sarcoidosis, with electrocardiographic abnormalities appearing in 50% of cases; [5] skin manifestations of cardiac sarcoidosis (such as erythema nodosum, granulomatous nodules, or papules) may be associated with heart block or cardiomyopathy. Patients with sarcoidosis may exhibit renal involvement, which is associated with abnormal calcium metabolism. Elevated vitamin D and calcium are found in the serum, and hypercalciuria may be found on urinary analysis.
斑块样结节病或冻疮样狼疮患者皮肤活检可能发现非干酪样肉芽肿（图示），而针对结节性红斑的活检则不然。应当进行胸片检查，因为肺和胸腔内淋巴结是结节病最常见的器官受累；胸片通畅发现双侧肺门淋巴结增大，肺组织正常。由于心脏结节病患者可能因室性心动过速或心动过缓导致猝死，大约50%的病例合并心电图异常，因此有指证进行24小时Holter监测；心脏结节病的皮肤表现（如结节性红斑，肉芽肿性结节或丘疹）可伴随心脏传导阻滞或心肌病。结节病患者可能出现肾脏受累，合并钙代谢异常。此时血清维生素D及钙水平升高，尿液检查发现高钙尿。
Image from Medscape.
Erythema marginatum is a flat to mildly elevated, pinkish, nonpruritic, transient eruption found primarily on the trunk and proximal extremities (arrow). It occurs in 10% of children with their first attack of acute rheumatic fever (ARF) [9](but overall, arises in less than 5% of patients with rheumatic fever). Subcutaneous nodules are also rare in rheumatic fever but are associated with more severe carditis and usually present many weeks after the onset of disease; they are generally found over bony prominences and are usually painless. Because it can involve the pericardium, epicardium, myocardium, and endocardium, ARF-associated carditis can be considered a pancarditis.
边缘性红斑为扁平至轻度高起的、粉色、非瘙痒性、一过性皮损，主要见于躯干及近端肢体（图示）。10%的急性风湿热患儿在初次发病时出现边缘性红斑（所有风湿热患者出现病例不超过5%）。皮下结节在风湿热罕见，但常见于更为严重的心脏炎，在发病后通常可持续数周；皮下结节常见于骨质突出部位，一般没有疼痛。由于急性风湿热可累及心包、心外膜、心肌和心内膜，因此，急性风湿热相关心脏炎可以认为是全心炎。
Image from Medscape.
This image depicts xanthelasma palpebrarum in a patient with hyperlipidemia. Xanthelasma are soft, yellow, cholesterol-filled plaques. Hyperlipidemia exists in approximately 50% of patients with xanthelasma palpebrarum, [10]yet xanthelasma palpebrarum is not indicative of an increased risk of cardiovascular disease. [11]
上图显示高脂血症患者的睑黄斑瘤。黄斑瘤为质地较软，黄色，且充满胆固醇的斑块。睑黄斑瘤患者约50%合并高脂血症，但睑黄斑瘤并不提示心血管疾病风险增加。
Image from Medscape.
In contrast, eruptive xanthomas (shown) usually appear when serum triglycerides exceed 1500 to 2000 mg/dL. At this level of triglyceridemia, chylomicronemia is present. Eruptive xanthomas are characterized by crops of 1- to 5-mm, yellow-orange papules with surrounding erythema, most commonly on the extensor surfaces of extremities and the buttocks. This condition is most strongly associated with hypertriglyceridemia types I, III, IV, and V. In general, when xanthomas are secondary to hyperlipidemia, these papules regress with correction of elevated lipids. Otherwise, treatment with cryotherapy or laser therapy may be warranted.
相反，发疹性黄色瘤（图示）常见于血清甘油三酯超过1500 - 2000 mg/dL的患者。在这种甘油三酯水平下，可出现乳糜血。发疹性黄色瘤的特点为聚集性出现的1-5 mm，黄色至橙色丘疹，周围有红斑，最常见于肢体伸展侧及臀部。发疹性黄色瘤与I、III、IV和V型高甘油三酯血症关系密切。总之，若黄色瘤继发于高脂血症，当纠正血脂水平后，这些丘疹也逐渐消退。另外，可以考虑冷冻或激光治疗。
Image courtesy of Medscape | Vibhuti N Singh, MD.
A patient with congestive heart failure is shown.
图为一名充血性心力衰竭患者。
Images courtesy of Andres Mora, MD.
问题3：血管的何种改变能够引起图中表现？
答案3：血管内静水压升高
Starling forces (the balance between hydrostatic and oncotic or colloid pressures) determine the amount of fluid in the interstitial space. Edema that results from changes in these forces is regarded as pitting edema (shown). An increase in hydrostatic pressure and/or a decrease in oncotic pressure within the vasculature would result in forcing fluid outward into the interstitium, whereas a relative decrease in hydrostatic pressure or increase in oncotic pressure would resorb fluid from the interstitial space or maintain fluid within the vasculature. Lymphatic obstruction may also cause edema, but it is generally unilateral, affecting only the side ipsilateral to the obstruction. [12]
Starling作用（静水压与胶体渗透压的平衡）决定了组织间液量。这些因素的改变能够导致可凹性水肿（图示）。血管静水压升高和（或）胶体渗透压降低可造成体液外渗进入组织间隙，而静水压降低或胶体渗透压升高则会从组织间隙重吸收液体或维持血管内液体。淋巴管阻塞也可以导致水肿，但通常为单侧，仅累及阻塞同侧。
Image courtesy of Carla G Arellano P, MD.
In addition to congestive heart failure, the differential diagnosis of edema should include venous insufficiency, which is the most common cause of lower extremity edema. Venous insufficiency also creates a symmetrical edema, but it may be associated with additional features, including varicose veins, leg discomfort, nonhealing ulcers, and lipodermatosclerosis (arrows).
除充血性心力衰竭外，水肿的鉴别诊断还包括静脉功能不全（下肢水肿最常见的原因）。静脉功能不全也可以引起对称性水肿，但可出现其他表现，包括静脉曲张，下肢不适，溃疡不愈合，以及脂性硬皮病（箭头）。
Lipodermatosclerosis is characterized by skin changes, including capillary proliferation, fat necrosis, and fibrosis of the skin and subcutaneous tissue. The skin turns reddish brown as a result of hemosiderin deposition from red blood cells. The pivotal finding of edema involving the ankles and feet indicates lymphatic obstruction instead of or in addition to venous insufficiency.
脂性硬皮病的特点为皮肤改变，包括毛细血管增生，脂肪坏死，以及皮肤和皮下组织纤维化。由于红细胞中的血铁黄素沉积，皮肤颜色变为棕红色。踝和足部水肿提示除静脉功能不全外，出现淋巴管阻塞。
Image from Medscape.
Metabolic syndrome is a risk factor for coronary heart disease, as well as diabetes, fatty liver, and several cancers. [13]
与糖尿病、脂肪肝和多种肿瘤相似，代谢综合征是冠心病的危险因素。
Image from Medscape.
问题4：代谢综合征的哪项诊断标准与图中箭头所示表现有关？
答案4：空腹血糖 ≥100 mg/dL
This image shows brown, velvety plaques with skin tags (arrow) in the axilla of a patient with acanthosis nigricans, a well-recognized cutaneous manifestation of insulin resistance. Although the exact mechanism has not been described, insulin or insulinlike growth factor likely induces epidermal cell propagation. Other factors that may play a role include epidermal growth factor and fibroblast growth factor receptors. These result in epidermal keratinocyte and dermal fibroblast proliferation, which manifests as acanthosis nigricans.
上图显示黑棘皮病患者腋窝出现的棕色、天鹅绒样斑块皮赘（箭头），这是胰岛素抵抗患者众所周知的皮肤表现。尽管确切机制尚不清楚，胰岛素或胰岛素样生长因子可能引起表皮细胞增生。其他致病因素包括表皮生长因子和成纤维细胞生长因子受体。这些因素导致表皮真核细胞及真皮成纤维细胞增生，表现为黑棘皮病。
Image courtesy of Dan Brito Guzman, MD.
This patient has a history of mechanical heart valve replacement and conjunctival pallor (shown).
此患者有机械瓣膜置换病史，患者睑结膜苍白。
Image courtesy of Dan Brito Guzman, MD, and Jean Bustamante, MD.
问题5：此患者外周血涂片可能看到以下哪种表现？
答案5：D. 裂细胞
Pallor in a patient with a prosthetic valve may be indicative of hemolytic anemia. In fact, 70% of prosthetic heart valve recipients develop low-grade hemolytic anemia, with the condition being severe in 3%. Among patients with caged-ball valves or perivalvular leaks, the incidence is increased. Because this anemia is hemolytic and microangiopathic, schistocytes (fragmented red blood cells, arrow) can be found on peripheral blood smear. The sheer force of red blood cells against the prosthetic valve results in physical damage and fragmentation. Jaundice is not observed, since hemolysis rarely results in a bilirubin level greater than 3 mg/dL
人工瓣膜置换术患者面色苍白可能提示溶血性贫血。事实上，70%的人工心脏瓣膜置换术患者发生低度溶血性贫血，3%为严重病例。在笼球瓣或瓣周漏患者，溶血性贫血发生率升高。由于贫血为溶血性及微血管性，因此外周血涂片可见裂细胞（红细胞碎片，箭头）。红细胞与人工瓣膜之间的剪切力导致物理损伤及碎片化。由于溶血很少导致胆红素水平超过3 mg/dL，因此观察不到黄疸的发生。
Image from Medscape.
Oral manifestations of Kawasaki disease, an acute febrile vasculitic syndrome of early childhood, include red lips and strawberry tongue (shown).
川崎病是一种儿童的急性发热性血管炎综合征，口部表现包括红色嘴唇及草莓舌（图示）。
问题6：川崎病最严重的并发症是什么？
答案6：冠脉动脉瘤
Image from Wikimedia Commons | mprice18.
Although most children with Kawasaki disease come to medical attention because of a high, unremitting fever, there are many cutaneous manifestations of the disease. Cardiovascular findings are not included in the diagnostic criteria but strongly support the diagnosis, since Kawasaki disease–like conditions do not have cardiac involvement. Prompt treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin is the current standard of care. Coronary aneurysms (shown) occur in 25% of patients not treated with IVIG, compared with approximately 1-5% of patients treated with it. [14] Aspirin has a synergistic effect with IVIG, so it is initiated at a high dose. Aspirin may be continued at a lower dose for its antiplatelet effects to further reduce the risk of thrombus formation.
尽管多数川崎病患儿因高热不缓解就诊，但本病还有很多皮肤表现。诊断标准中并未包括心血管表现，但一旦出现，则强烈支持川崎病的诊断，因为与川崎病相似的其他疾病并不累及心脏。立即给予静脉丙种球蛋白(IVIG)及大剂量阿斯匹林治疗。未接受IVIG治疗的患者25%发生冠脉动脉瘤（图示），接受治疗者发生率仅为1-5%。阿斯匹林与IVIG有协同效应，因此初始应使用大剂量。因为阿斯匹林有抗血小板作用，能够进一步降低血栓形成的风险，因此应使用小剂量维持。
Image from Medscape.
Other common findings in the acute phase of Kawasaki disease include fever, nonexudative bilateral conjunctivitis (90%), anterior uveitis (70%), perianal erythema (70%), lymphadenopathy, strawberry tongue, and erythema and edema of the hands and feet. [15] Findings in the subacute phase (once fever begins subsiding) include desquamation of the digits (shown) and thrombocytosis. During this phase, a coronary aneurysm may develop and the risk of sudden death is highest.
川崎病急性期最常见的其他表现包括发热，非渗出性双侧结膜炎(90%)，前葡萄膜炎(70%)，肛周红斑(70%)，淋巴结增大，草莓舌，以及手足红斑和水肿。亚急性期（一旦发热开始消退）表现包括手指脱屑（图示）及血小板增多。在这一阶段，可发生冠脉动脉瘤，猝死风险最高。
Image courtesy of Dirk M. Elston, MD.
This 25-year-old patient presented with an acute onset of fever and chills, a heart murmur, and nontender maculae on the toes (shown), soles of the feet, fingers, and palms of the hands.
一名25岁患者出现急性发热及寒战，心脏杂音，及脚趾（图示）、足底、手指和手掌等部位非痛性斑疹。
What organism is most likely to be found in his blood?
A. Streptococcus pyogenes
B. Streptococcus viridans
C. Streptococcus bovis
D. Staphylococcus aureus
E. Haemophilus influenzae
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