Left Atrial Myxoma Resenting with Migratory Erythematous Maculopapules: DISCUSSION

Cardiac myxomas are the most prevalent primary cardiac tumors, although they are rare. While they arise in any of the four heart chambers, or rarely on heart valves, about 90% are located in the atria, with a left-to-right ratio of approximately 4:1. The common clinical manifestations of cardiac myxomas have included obstructive, embolic and constitu­tional symptoms and signs such as fever and malaise. Obstruction of intracardiac blood flow by a left atrial myxoma may cause congestive heart failure, chest pain, syncope, or a murmur. Constitutional symp­toms have been estimated to occur in 90% of patients with cardiac myxoma and were charac­terized by fever, malaise, arthralgia, weight loss, anemia, elevated ESR, and elevated gamma globulin. Several studies demonstrate a correlation between interleukin-6 production by cardiac myxomas and systemic inflammatory findings. Embolism occurs in 20% to 45% of patients with cardiac myxoma, and approximately half of all myxomatous emboli from the left cardiac myxoma go to the brain. Tumor embolization caused by left atrial myxomas may present the first symptomatic manifestation, which may be multiple and massive. The onset of a neurological deficit may be gradual or sudden. The extremities are the next most common sites of embolic phenomenon. Our patient had migratory maculopapules as the sole manifestation without any cardiac or neurological symptoms.

Cutaneous manifestations of cardiac myxoma are usually nonspecific. They may be due to embolism from the myxoma, but there are also other none- mbolic cutaneous signs. An association between nonembolic cutaneous lesions and cardiac myxomas was first reported in a patient with prominent facial lentiginosis and a left atrial myxoma. Subsequent reports have described the NAME syndrome (nevus, atrial myxoma, myxoid neurofibromas, and epheli- des) and the LAMB syndrome (lentigines, atrial myxoma, blue nevi). Approximately 10% of pati­ents with myxoma have a familial cardiac myxoma syndrome, known as Carney syndrome, characterized by autosomal dominant transmission, multiple car­diac and often extracardiac myxomas, spotty pig­mentation, and endocrine overactivity. Our patient had no familial history of cardiac myxoma and had a small number of lentigines on the face, fewer than the number described in reported cases, so a diagnosis of LAMB syndrome or Carney syndrome could not be established.
The cutaneous manifestations of emboli from atrial myxomas include erythematous macules and papules mainly acral in location, digital cyanosis, petechiae, splinter hemorrhage, telangiectasia, livedo reticularis, Raynaud’s phenomenon, ulcerating lesi­ons, and a reddish-violet malar flush. In our patient, the pattern of the cutaneous findings was similar to that described by Garcia-F-Villalta et al. In their report, the patient had noted five or six episodes of acral papular erythematous lesions mainly on the lower extremities and ischemic neurological events with disorientation, dysarthria, and right arm weak­ness. There were some other reports showing erythe­matous maculopapules with neurologic manifesta­tions in the English literature. Al-Mateen et al. also described two children with cerebrovas­cular events caused by emboli from left atrial myxomas. They described transient cutaneous erup­tions involving the extremities as ‘red spots’. The red spots were noted before the onset of the cerebral ischemic event, therefore cutaneous findings would be an important clue in prompt diagnosis and early intervention.

Because there is a broad spectrum of cutaneous findings in patients with cardiac myxoma and skin lesions may be nonspecific, the diagnosis of cardiac myxoma may be difficult, especially when cardiac symptoms are not present. However, cutaneous findings might be important clues to the diagnosis as in our case, so the dermatologist must first be aware of the multitude of cutaneous findings asso­ciated with this tumor.

Category: Disease

Tags: Atrial myxoma, Cutaneous embolism

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