Key result
A 12-year-old boy initially presenting with cryoglobulinemic vasculitis was diagnosed with Carney Complex after developing a stroke and an obstructive left atrial myxoma.
Case Report (n=1)
This case highlights that cryoglobulinemic vasculitis can be an unusual preceding presentation of Carney Complex before the discovery of a cardiac myxoma.
May precede myxoma in Carney complex; single case leaves association hypothesis-generating.
Carney complex (CNC) is a disorder characterized by skin pigmentary abnormalities and benign cardiac, endocrine, skin and neuronal tumors. We present a previously healthy 12-year-old boy with recurrent pain and swelling of the feet. One year later he presented with stiffness of the fingers and the toes were painful with no swelling. Rheumatoid factor, ANCA and ANA were all negative but cryoglobulins were positive. Unusual lentigines were noted. The monthly attacks with pain and joint swelling were treated sufficiently with prednisolone and azathioprine. Two years later he was admitted with classical symptoms of a stroke. MRI revealed small middle cerebral artery infarctions but no signs of schwannomas or vasculitis on the angiography. An echocardiography revealed an obstructive tumor in the left atrium, 5-6 centimeters in diameter, diagnosed as myxoma, which was surgically removed. The lentigines and myxoma led to the suggestion of Carney Complex and the diagnosis was confirmed by a PRKAR1A mutation found in the child as well as the mother.
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Glerup et al. (2014) conducted a case report in Carney complex and cryoglobulinemic vasculitis (n=1). A 12-year-old boy initially presenting with cryoglobulinemic vasculitis was diagnosed with Carney Complex after developing a stroke and an obstructive left atrial myxoma.
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