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Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022
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Image from Palmucci S, Torrisi SE, Caltabiano DC, et al. Insights Imaging. 2016 Aug;7(4):571-87. [Open access.] PMID: 27222055, PMCID: PMC4956623. [Creative Commons Attribution 4.0 International License (CC by 4.0).]
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Patients with cardiovascular disease often present with manifestations on the skin. The above image, for example, shows several patients with sarcoidosis—a multisystem, inflammatory disease—presenting with (a) lesions, (b) papules, (c) plaques, and (d) lupus pernio (a form of cutaneous sarcoidosis). Cutaneous signs such as these can play a valuable role in guiding healthcare providers 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 courtesy of Medscape.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Livedo reticularis, shown here on the plantar surface of the feet, is the most common dermatologic manifestation of cholesterol embolism, 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.
Which of the following statements is true regarding the diagnosis of cholesterol embolism?
Cholesterol embolism should be highly suspected in patients with known atherosclerotic disease and a classic history of renal failure, abdominal pain, and livedo reticularis.
Pedal pulses may be decreased bilaterally and play no role in the diagnosis.
Laboratory testing shows a normal white blood cell (WBC) count and erythrocyte sedimentation rate (ESR) and normal C-reactive protein (CRP) and fibrinogen levels.
None of the above statements are true for the diagnosis of cholesterol embolism.
Adapted image from Hsien YM, Mustapha M, Hamzah JC, Maskon O, Ken CC, Hamdi CH. BMC Ophthalmol. 2016;16:32. [Open access.] PMID: 27013074; PMCID: PMC4807534. [CC by 4.0.]
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Answer: A. Cholesterol embolism should be highly suspected in patients with known atherosclerotic disease and a classic history of renal failure, abdominal pain, and livedo reticularis.
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 (arrow) 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 WBC count, a decreased red blood cell (RBC) count, and elevated inflammatory markers, such as CRP, ESR, and fibrinogen.[4]
Image courtesy of Medscape.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
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 take caution or avoid biopsy in the presence of obvious ischemia. 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 courtesy of Medscape.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Sarcoidosis—a multisystem inflammatory disease of unknown etiology characterized by noncaseating granulomas occurring in organs and tissue—manifests itself on the skin in 25% of patients. Plaque sarcoidosis (shown) features round to oval and red-brown to purple lesions that 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 frequently involved. Plaque sarcoidosis generally has a chronic cutaneous manifestation (>2 years) associated with more severe systemic involvement.[6]
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 Sand M, Sand D, Thrandorf C, Paech V, Altmeyer P, Bechara FG. Head Face Med. 2010;6:7. [Open access.] PMID: 20525327; PMCID: PMC2903548. [Creative Commons Attribution 2.0 Generic License (CC by 2.0).]
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
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]
Which of the following should be included in the workup for suspected sarcoidosis?
Tissue biopsy
Chest radiography
24-hour Holter monitoring
Basic metabolic profile, including serum calcium, and urine analysis
All of the above
Image courtesy of Medscape.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Answer: E. All of the above
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, as can complete heart block, 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 infiltrative 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 courtesy of Medscape.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
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 <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.
Top image courtesy of Medscape. Bottom image courtesy of Duke University Medical Center.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
The top 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 alone is not indicative of an increased risk of cardiovascular disease.[11]
In contrast, eruptive xanthomas (bottom image) 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.
Image courtesy of Medscape | Vibhuti N Singh, MD.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
The legs of a patient with congestive heart failure are shown.
What changes occur in the vasculature to cause the phenomenon displayed?
Increased oncotic pressure within the vasculature
Increased hydrostatic pressure within the vasculature
Lymphatic obstruction
None of the above
Images courtesy of Andres Mora, MD.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Answer: B. Increased hydrostatic pressure within the vasculature
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.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
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 RBCs. The pivotal finding of edema involving the ankles and feet indicates lymphatic obstruction instead of or in addition to venous insufficiency.
Image courtesy of Medscape.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Oral manifestations of Kawasaki disease, an acute febrile vasculitic syndrome of early childhood, include red lips and strawberry tongue (shown).
What is the most serious complication of Kawasaki disease?
Coronary artery disease
Coronary artery fistula
Coronary artery aneurysm
Cardiomyopathy
Angiography images in a 6-year-old patient of a totally occluded middle right coronary artery with a dilated aneurysm (left) and of a patent left main coronary artery and left circumflex artery (right) from Hsu YC, Liang KW, Lin MC, Fu YC, Jan SL. J Med Case Rep. 2012;6:111. [Open access.] PMID: 22507194; PMCID: PMC3349492. [CC by 2.0.]
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Answer: C. Coronary artery aneurysm
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, because 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 who receive it.[13] 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 courtesy of Dirk M. Elston, MD.
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
A 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?
Streptococcus pyogenes
Streptococcus viridans
Streptococcus bovis
Staphylococcus aureus
Haemophilus influenzae
Image of Osler nodes on the hand of a 43-year-old male from Wikimedia Commons | Roberto J Galindo. [CC by 4.0.]
Dermatologic Hints to Hidden Heart Disease
Rashid M Rashid, MD, PhD; Brittany Sambrano Barros, MD | November 14, 2022 | Contributor Information
Answer: D. Staphylococcus aureus
In a young patient with presumably normal heart valves, acute onset of fever and chills, heart murmur, and Janeway lesions (shown on the previous slide) or Osler nodes (shown above), infective endocarditis is the most likely diagnosis.
Janeway lesions are associated with acute endocarditis, of which S aureus is the most common cause. Osler nodes are associated with subacute bacterial endocarditis and S viridans. Osler nodes are painful, erythematous nodules most commonly found on the pulp of fingers and toes.
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Contributor Information
Authors
Rashid M Rashid, MD, PhD
Director
Mosaic Dermatology Houston
Houston, TX
Disclosure: Rashid M Rashid, MD, PhD, has disclosed no relevant financial relationships.
Disclosure: Brittany Sambrano Barros, MD, has disclosed no relevant financial relationships.
Reviewer
Yasmine S. Ali, MD, MSCI, FACC, FACP
President, LastSky Writing, LLC;
Assistant Clinical Professor of Medicine
Vanderbilt University School of Medicine;
Chief Editor, Atherosclerosis and Congenital Heart Disease sections
Medscape Drugs and Diseases from WebMD
Disclosure: Yasmine S. Ali, MD, MSCI, FACC, FACP, has disclosed the following financial relationships: President, LastSky Writing, LLC; Consultant, Philips Healthcare, GE Healthcare, M Health, Athena Health, Corvidane, About, Inc, Remedy Health Media, and Elsevier.
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References
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McGevna LF. Cutaneous manifestations of cholesterol embolism. Medscape Drugs & Diseases from WebMD. Updated: August 26, 2021. Available at: https://emedicine.medscape.com/article/1096593-overview. Accessed October 20, 2022.
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