Stressed Out About Earlobe Creases

Figure 10-34: Hyperlipidemia: xanthomata associated with coronary artery disease. A. On the extensor tendons of the hand. B. On the Achilles tendon (arrow).

In Werners syndrome, the skin is tightly stretched over the underlying bones.112 There is marked loss of subcutaneous tissue, and ulcerations occur over the legs. Severe coronary atherosclerosis often results in MI at an early age. Physical findings in diabetes mellitus may include tight skin and necrobiosis diabeticorum, an atrophy of the skin of the lower extremities characterized by ovoid plaques with central telangectasia and a violet, undurated perimeter. Progeria is a rare disorder in which the face is small and prematurely aged, the eyes bulge, and the nose is beaked. Severe atherosclerosis with early MI is a common cause of death in early life.113 A diagonal earlobe crease and short tufts of ear-canal hair curiously have been associated with coronary artherosclerosis.114 There is a modest correlation between male-pattern baldness involving the vertex of the scalp in men under 55 years of age and an increased risk of MI.115 Patients resemble those with Marfan's syndrome because they have long extremities, pectus carinatum, and kyphoscoliosis. Pseudoxanthoma elasticum has been associated with fibrosis of the coronary artery and calcification of peripheral arteries116 (see Chap. 76). A glycosphingolipid is deposited in the arterial endothelium of patients with Fabry's disease and may result in angina pectoris or MI. Patients with Hurler's syndrome have mental retardation, a large, boat-shaped head, a broad nose, large lips, small, widely spaced teeth, and a large, protuberant tongue. Glycosaminoglycan deposition in the coronary arteries is present.117 Myocardial fibrosis due to repeated coronary small-vessel spasm has been postulated to be a result of progressive systemic sclerosis.81

Vasculitis may be due to SLE, rheumatoid arthritis, Behget's disease,108 Kawasaki's disease, and polyarteritis. Cutaneous infarction, nodules, petechiae, livedo reticularis, gangrenous digits, MI, heart failure, and hypertension may be due to polyarteritis118 (see Chap. 76).

Arteriovenous shunts may be found in extensive skin disease, hereditary hemorrhagic telangiectasia, and the Klippel-Trenaunay-Weber syndrome. Kaposi's sarcoma or exfoliative dermatitis due to psoriasis may divert the blood supply through shunts in the skin to produce highoutput cardiac failure. Clues to underlying arteriovenous fistula as a cause of high-output failure include a barely discernible scar from a knife wound or a surgical incision. Telangiectasias of the fingertips, face, palate, lips, and tongue, as well as pulmonary and hepatic arteriovenous fistulas, are components of hereditary hemorrhagic telangiectasia.M The triad of anomalies that Klippel-Trenaunay-Weber syndrome comprises are vascular nevus, large varices, and bony or soft tissue hypertrophy.119 Marked enlargement of a limb(s) and facial hemihypertrophy are features of this disorder, in which part or all of the deep venous system is absent and arteriovenous malformation is often present. Hemangiomas of the skin also may indicate multinodular hemangiomatosis of the liver, a cause of high-output heart failure in infancy (Fig. 10-35).

High Output Heart Failure
Figure 10-35: Klippel-Trenaunay syndrome: hypertrophy of left side of face and tongue in a patient with port-wine stains, gigantism of digits, and varicose veins.

Stenosis of large arteries may occur with supravalvular aortic stenosis, rubella syndrome, Turner's syndrome, and neurofibromatosis. The face of a child with supravalvular aortic stenosis (Williams syndrome) is almost diagnostic (Fig. 10-36). The head is small, with an elflike appearance; the cheeks are full and baggy; and the mouth is large.120 Thick lips and peg-shaped, widely spaced teeth are typical findings. The forehead is prominent and broad. Mental retardation is often present. The supravalvular aortic stenosis may be a localized ridge or a diffuse narrowing of the aorta beginning just above the sinuses of Valsalva. Pulmonic artery branch stenosis is frequently present. Coarctation of the aorta is a common cardiac lesion in Turner's syndrome,61 and neurofibromatosis has been associated with renal artery stenosis.

Marked Jugular Venous Distention
Figure 10-36: Supravalvular aortic stenosis: turned-up nose, broad cheeks, large mouth with peg-shaped teeth, and large ears.

Facial swelling and jugular venous distention may be early signs of superior vena caval obstruction from clot or tumor.

Miscellaneous Disorders

Multiple lentigines, cutaneous myxomas, myxoid fibroadenomas of the breast, and various endocrine abnormalities are features of a recently described inherited disorder in which single or multiple cardiac myxomas occur.121 Telangiectasia of the tongue and lips or under the fingernails may be associated with a pulmonary arteriovenous fistula (Figs. 10-37 and 10-38, Plates 39 and 40). A susceptibility to atrial fibrillation and atrial flutter has been documented in patients who have facioscapulohumeral muscular dystrophy.122 Sinus node dysfunction, elbow contractures, and humeroperoneal weakness are manifestations of Emery-Dreifuss muscular dystrophy.123

Focal Telangiectasia Face Skin Tumours

Figure 10-37: (Plate 39) Hereditary hemorrhagic telangiectasia. Telangiectasia under nails. (From Silverman ME, Hurst JW. The hand and the heart. Am J Cardiol 1968; 22:609. Used with permission from the publisher.)

Pulmonary Arteriovenous Fistula
Figure 10-38: (Plate 40) Hereditary hemorrhagic telangiectasia. Telangiectasia of tongue and lips may be associated with a pulmonary arteriovenous fistula.

Single or multiple rhabdomyomas may develop within the myocardium and cause heart failure, valvular obstruction, or arrhythmias in patients with tuberous sclerosis!24 (Fig. 10-39, Plate 41). The diagnosis is suggested by the presence of yellow-brown angiofibromas (adenoma sebaceum) on the face, subungual fibromas around the fingernail, cafe-au-lait spots, and subcutaneous nodules. Finally, horizontal ear creases often are associated with the presence of extensive CAD (Fig. 10-40, Plate 42).

Adenoma Sebaticum
Figure 10-39: (Plate 41) Tuberous sclerosis. Adenoma sebaceum may be associated with rhabdomyomas of the myocardium.
Image Adenoma Sebaceum
Figure 10-40: (Plate 42) Horizontal ear creases often are associated with the presence of extensive CAD.


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