The clinical features of cardiac myxoma are based on its location, size, and mobility and can be morphologically variable. However, almost patients with this condition present with one or more clinical manifestations of the classic triad: 1) intracardiac obstructive signs; 2) extracardiac embolic signs; or 3) constitutional or systemic manifestations1
First, intracardiac obstructive signs occurred in 67% of cardiac myxoma patients4
. Progressive congestive heart failure (dyspnea, orthopnea) was the most common symptom (64%). Second, extracardiac embolic signs were seen in about one-third of patients. Approximately half of all embolic sites were within the central nervous system. Third, systemic symptoms, including fever, myalgia, arthralgia, or Raynaud's phenomenon, were present in 90% of patients5
Cutaneous aspects of cardiac myxoma are usually non-specific and can be classified into 3 subtypes: 1) embolic cutaneous signs; 2) non-embolic cutaneous signs associated with auto-immune symptoms; and 3) cutaneous signs of a complex syndrome. First, embolic cutaneous signs resulting from fragmentation or complete tumor detachment occur in approximately 30~40% of cases. Tough myxomatous embolisms, which can affect any part of the arterial bed and involve the cerebral circulation in about 50% of cases, result in cerebrovascular accidents. In addition, embolization to the lungs, coronary arteries, kidneys, spleen, intestines, and extremities also occurs. According to Pinede's study, peripheral (limb) embolizations were present in 13% of patients4
. They may appear as acral erythematous papular eruptions, telangiectasia, petechiae, splinter hemorrhages, and livedo reticularis3
and be episodic or recurrent. Sometimes they may progress into digital cyanosis and necrosis as a result of an ischemic condition caused by obstruction of blood circulation resulting from embolization. The histopathological findings were based on the presence of characteristic myxoma cells surrounded by an amorphous myxoid matrix that consisted of an acid-mucopolysaccharide-rich stroma. In our patient, the postoperative specimen showed myxoma cells scattered throughout the matrix that had a spindle or stellate shape, an ovoid nucleus, and an eosinophilic cytoplasm. These features disappear when the tumor is eliminated6
Second, cutaneous features may be associated with autoimmune symptoms including Raynaud's phenomenon, malar erythema, and vasculitis, which can be explained by myoxomal secretion of interleukin-67
. These symptoms usually disappear after removal of the cardiac myxoma, although prolonged elevation of antinuclear antibodies has been noted8
Third, some cutaneous findings suggest the diagnosis of cardiac myxoma as part of a more complex syndrome. Nevus, atrial myxoma, myxoid neurofibromas, and ephelides as well as lentigines, atrial myxoma, and blue nevi have been described9
, and a subsequent report has described Carney syndrome, which is characterized by autosomal dominant transmission, myxoma, spotty pigmentation, endocrine overactivity, and schwannomas10
The differential diagnosis of the cutaneous findings of cardiac myxoma consists of several diseases including vasculitis, livedo reticularis, and pigmented purpuric dermatosis.
To the best of our knowledge, 4 cases of cardiac myxoma associated with cutaneous manifestations have been reported in the Korean medical literature3,9,11,12
. Of these 4 previously described patients, one was related to migratory erythematous maculopapules, another was associated with pseudovasculitis, and the others presented with livedo reticularis.
Because the tumor's histological benignancy contrasts with its malignant clinical behavior in terms of systemic embolization, early diagnosis and surgical intervention are essential. However, cardiac myxoma illustrates that this benign tumor may cause a broad spectrum of clinical manifestations including not only cardiac disease but also infective, immunologic, or neurologic diseases. Also, it has no specific or pathognomic signs or symptoms. Therefore, clinical diagnosis of cardiac myxoma remains difficult, especially when cardiac symptoms are not present as in our patient. In this case, the skin lesions were the first sign of the disease and may play a very important role in the diagnosis of a patient with cardiac myxoma.
In conclusion, this case of cardiac myxoma diagnosed by the presence of purpuric macules on both palms and soles emphasizes to clinicians that cutaneous manifestation can be an important clue in the prompt diagnosis and early intervention. Therefore, although it is uncommon, cardiac myxoma should be considered when the cutaneous findings described above are observed in a patient, so dermatologists must recognize the various cutaneous findings associated with it.