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[MEDSCAPE]: 隐匿性心脏病:你应当了解的19种皮肤表现(7/9)
2017年07月13日 临床话题, 基本知识 暂无评论

Hidden Heart Disease: 19 Dermatologic Clues You Should Know

Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | June 13, 2017

Image from Medscape.

Patients with cardiac disease often present with manifestations on the skin. The above image, for example, shows a patient with sarcoidosis—a multisystem, inflammatory disease—presenting with lupus pernio, appearing as nodules on the nasal tip and sidewall. Cutaneous signs such as this can play a valuable role in guiding physicians to the correct cardiac diagnosis. Review our slideshow to check your knowledge of dermatologic findings that should alert the clinician to further investigate the cardiovascular system.


Image from Medscape.

Skin findings are the most common clinical sign of a cholesterol embolism; they are present in about 34% of patients, according to one systematic review. [1] Livedo reticularis, shown here on the plantar surface of the feet, is the most common dermatologic manifestation of cholesterol embolism, being found in 16% of patients who have this condition.[1] It is characterized by mottled, erythematous discoloration of the skin, which blanches on pressure. A painful, cyanotic toe is found in over one quarter of cholesterol embolism cases.


Image from Medscape.


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]


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.)


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]


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.


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]


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]


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.


Image from Medscape.


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.


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.


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]


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.


Images courtesy of Andres Mora, MD.



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]


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).


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.


Image from Medscape.

Metabolic syndrome is a risk factor for coronary heart disease, as well as diabetes, fatty liver, and several cancers. [13]


Image from Medscape.


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.


Image courtesy of Dan Brito Guzman, MD.

This patient has a history of mechanical heart valve replacement and conjunctival pallor (shown).


Image courtesy of Dan Brito Guzman, MD, and Jean Bustamante, MD.


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).




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.


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.


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.


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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