A number of multisystem disorders can affect both the heart and the skin, and cutaneous examination often provides diagnostic clues to such entities. Recognition of the multisystem aspects of these conditions by both dermatologists and cardiologists is imperative for proper diagnosis and management. Awareness of potential cutaneous complications of cardiac therapies is also essential for the dermatologist. Therapies directed at improvement of muscle function (e.g., digoxin) rarely result in cutaneous disease, while therapies seeking to achieve fluid reduction (diuretics), afterload reduction, and control of rhythmic disturbances can affect the skin. In addition, increasingly frequent use of potent immunosuppressive agents for autoimmune cardiovascular disease and cardiac transplantation may lead to a variety of dermatologic manifestations, including an increased risk of skin cancers, again emphasizing the need for interspecialty collaboration.

This text reviews several multisystem disorders with associated cardiovascular abnormalities. Table 37-1 lists cardiac abnormalities associated with these multisystem disorders, Table 37-2 lists common dermatological findings associated with primary cardiovascular disorders, and common cutaneous side effects of cardiac medications are described in Table 37-3 .

TABLE 37-1

Cardiac Manifestations in Multisystem Disorders with Prominent Cutaneous Features

Disease	Cardiac Manifestation	Cutaneous Features	Comments
Primary systemic amyloidosis	Congestive heart failure, conduction disturbances, cardiomegaly	Pinch purpura, waxy translucent papules or diffuse waxy skin infiltration, enlarged tongue, hemorrhagic bullae	Due to immunoglobulin light chains; associated with nonprogressive plasma cell dyscrasia and myeloma, as well as renal and neurologic involvement
Behçet’s disease	Pericarditis, conduction abnormalities, valvular disease, coronary arteritis, myocarditis. Recurrent thromboses	Oral and genital aphthae, pathergy, pustular vasculitis, pyoderma gangrenosum-like lesions, sterile vesicopustules, erythema nodosum, superficial thrombophlebitis	Ocular involvement (leading cause of morbidity), arthritis, central nervous system (CNS) disease
			Inflammatory bowel disease can share many features and should be excluded
Carcinoid syndrome	Endocardial plaque—tricuspid insufficiency, pulmonary stenosis, right-sided heart failure.	Flushing, telangiectases, sclerodermoid features may occur as a late manifestation	Serotonin-producing tumor, most commonly of the intestine. Usually metastatic to the liver prior to symptom onset
	Left-sided cardiac manifestations exceedingly rare due to pulmonary deactivation of vasoactive substances
Cardiofaciocutaneous syndrome	Sparse, curly, woolly, or brittle hair, ichthyotic skin	Pulmonary stenosis, atrial septal defect, hypertrophic cardiomyopathy	Many other associated cutaneous findings such as keratosis pilaris, palmoplantar keratoderma, café-au-lait macules
Carney complex (including NAME and LAMB syndromes)	Atrial myxoma	Cutaneous myxomas and lentigines	Carney’s includes endocrine neoplasia of the adrenal, pituitary, and/or testes. Mutations in the PRKRA1A gene have been identified in many patients with familial cardiac myxomas
Cushing’s syndrome	Hypertension	Atrophy and striae, ecchymoses, acne, telangiectases	Due to overproduction of cortisol, commonly iatrogenic
Cutis laxa	Aortic dilation and rupture, pulmonary artery stenosis, right-sided heart failure	Looseness of the skin, premature aging appearance. Skin findings may be present from birth	Dominant (OMIM#123700), recessive (OMIM#219100), and X-linked (OMIM#304150) forms exist. Acquired forms also exist
Dermatomyositis	Cardiac arrhythmias, including atrial fibrillation/flutter, congestive heart failure, coronary artery disease	Gottron’s papules, heliotrope rash, photodistributed poikiloderma, nail fold capillary changes	Clinically evident cardiac involvement is a poor prognostic sign
Diabetes mellitus	Coronary artery and peripheral vascular disease	See Chapter 24	—
Down syndrome	Septal defects, patent ductus arteriosus, tetralogy of Fallot	Palmoplantar hyperkeratosis, alopecia areata, cutis marmorata, fissured tongue	Characteristic dysmorphic features such as upslanting palpebral fissures, epicanthic folds, transverse palmar crease
Ehlers–Danlos syndrome	Aortic and pulmonary artery dilation, mitral and tricuspid valve prolapse, arterial rupture	Hyperelasticity of the skin, “cigarette paper” scars, ecchymoses	Cardiac diseases is limited to classic (OMIM#130000), hypermobility (OMIM#130020) and vascular (OMIM#130050) types
Endocarditis—bacterial or fungal	Vegetation and dysfunction of the valves, can lead to myocardial abscess and heart failure	Purpura, splinter hemorrhages (linear purpura in nail beds), Janeway lesions (nontender macules on palms and soles), Osler’s nodules (tender subcutaneous nodules usually on distal digits)	Fever.
			Roth’s spots (retinal hemorrhages).
			May simulate vasculitis
Exfoliative erythroderma	High-output cardiac failure	Exfoliative diffuse dermatitis	The eruption may be due to eczematous or atopic dermatitis, psoriasis, cutaneous T-cell lymphoma, drug eruption or other causes
Fabry’s disease	Mitral valve prolapse, conduction defects, congestive heart failure, myocardial infarction, cerebrovascular accidents	Angiokeratoma corporis diffusum, which may be an early feature and lead to diagnosis	Alpha-galactosidase A deficiency, X-linked (OMIM#301500), gene map locus Xq22. Renal failure is the usual cause of death
Hemochromatosis	Congestive heart failure, supraventricular arrhythmias	Generalized bronze hyperpigmentation	Diabetes, cirrhosis
Hyperlipidemias	Coronary artery disease	Xanthomas of all types	__
Kawasaki disease (mucocutaneous lymph node syndrome)	Coronary artery aneurysms are the major complication, coronary arteritis, valvular insufficiency, pericardial effusion may also occur	Glossitis and cheilitis, acral edema, desquamative erythema of the perineum, diffuse morbilliform eruption, conjunctival injection	High fever, lymphadenopathy, treatment with intravenous immune globulin can be beneficial.
			Infants <1 year old have highest risk of cardiac disease
Multiple lentigines (LEOPARD) syndrome	Electrocardiogram abnormalities, hypertrophic cardiomyopathy	Multiple lentigines
Loeys–Dietz syndrome	Arterial tortuosity and aneurysm	Velvety or translucent skin in some patients	Described in 2005. Caused by heterozygous mutations in genes encoding transforming growth factor-β receptors 1 and 2; other features include hypertelorism, and bifid uvula or cleft palate. Shares features with Marfan’s syndrome and vascular-type Ehlers–Danlos syndrome, but Loeys–Dietz patients lack joint hypermobility and can often be successfully treated with vascular surgery
Lyme disease	Heart block, myopericarditis	Erythema migrans, borrelial lymphocytoma in some cases in Europe	Multisystem disease divided into stages: early localized, early disseminated, and late
Multicentric reticulohistiocytosis	Pericarditis, myocarditis, congestive heart failure	Erythematous nodules of the hands and occasionally the face	Deforming arthritis is frequent
Neonatal lupus erythematosus (NLE)	Congenital heart block	Transient, photosensitive, nonscarring lesions of lupus erythematosus (SCLE-like), predilection for face and periorbital skin. May be first noted after phototherapy for neonatal jaundice. Resolve with dyspigmentation	Presumed to be due to transplacental passage of autoantibodies, most commonly Ro (SS-A). May have transient cytopenias, hepatitis. Mothers may be asymptomatic. Hydroxychloroquine may help prevent against NLE in subsequent pregnancies, but data are limited
Neurofibromatosis	Hypertension due to pheochromocytoma	Café-au-lait macules, neurofibromas, axillary freckling	—
Pseudoxanthoma elasticum	Premature atherosclerotic vascular disease, hypertension	Yellow papules on intertriginous surfaces, redundant lax skin	Upper or lower gastrointestinal hemorrhage. Angioid streaks in the eye, uterine hemorrhage. Autosomal dominant and recessive variants (OMIM#264800 and #177850)
Psoriasis	Increased risk of myocardial infarction and coronary artery disease	Well-demarcated erythematous plaques with micaceous scale, often involving knees, elbows, umbilicus, gluteal cleft, scalp	Associated with metabolic syndrome, obesity, diabetes mellitus, hyperlipidemia, smoking
Relapsing polychondritis	Aortic insufficiency, dissecting aortic aneurysm, valvular disease, arrhythmias	Beefy, red ears or other cartilaginous areas. Late cauliflower-ear deformity or other cartilaginous destruction	Arthritis, tracheal collapse. Dapsone may be helpful. Corticosteroids and/or other immunosuppressive therapies may also be beneficial
Rheumatic fever	Pancarditis in the acute phase. Late manifestations include mitral and/or aortic valve dysfunction	Erythema marginatum, subcutaneous nodules	Rare in United States. Follows pharyngitis due to group A β-hemolytic streptococcal infection. Polyarthritis, chorea, fever
Sarcoidosis	Conduction defects, congestive heart failure	Granulomatous papules, nodules, and plaques, often with a predilection for scars or tattoos. Subcutaneous nodules may also occur. Nonspecific lesions such as erythema nodosum may occur; erythema nodosum associated with better prognosis	Pulmonary disease, hypercalcemia, lymphadenopathy, hepatic, neurologic, and ocular involvement. Cardiac involvement denotes a poor prognosis and can lead to sudden death. Electrocardiogram recommended at diagnosis in all patients, echocardiogram and 24-h Holter monitor in patients with palpitations
Scleroderma	Conduction defects, pulmonary hypertension, pericarditis	Cutaneous sclerosis, Raynaud’s phenomenon	Cardiac involvement denotes a poor prognosis
Syphilis	Aortitis, especially of the ascending aorta	Multiple potential skin lesions including genital chancre in primary disease, diffuse papulosquamous eruption involving the palms and soles, alopecia, condyloma lata, mucous patches in secondary disease
Systemic lupus erythematosus	Verrucous endocarditis, pericarditis, coronary artery disease	Malar erythema, photosensitivity, lupus-specific skin lesions such as discoid and subacute cutaneous lupus	Anticardiolipin antibody may play a role in cardiac disease. Corticosteroid therapy may predispose to coronary artery disease
Tuberous sclerosis	Cardiac rhabdomyomas	Adenoma sebaceum, periungual and subungual fibromas, ash leaf macule, shagreen patch, fibrous forehead plaque	Renal and retinal hamartomas, CNS tumors, mental retardation, seizures, pulmonary lymphangioleiomyomatosis
Turner syndrome (gonadal dysgenesis)	Aortic coarctation	Alopecia of frontal scalp, webbed neck, short stature, koilonychia, numerous nevi	Despite multiple nevi, melanoma risk appears low.
			Increased risk of thyroid disease.
			May also be associated with increased incidence of alopecia areata and halo nevi
Thyroid disorders	Arrhythmias, palpitations, cardiomyopathy	Myxedema, ocular proptosis in Grave’s disease, pruritus
Vasculitis	Coronary artery vasculitis	Palpable purpura, nodules, livedo reticularis, ulcerations	Arthritis, gastrointestinal colic or bleeding, cardiac involvement is uncommon
Werner’s syndrome	Premature atherosclerosis	Premature graying, alopecia, sclerodermoid changes, loss of subcutaneous fat, ankle ulcerations	Myocardial infarction is usually responsible for death by the fifth decade. Autosomal recessive (OMIM#277700, gene map locus 8p12-p11.2). Other features include cataracts, malignancy

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