Toe Necrosis and Acute Myocardial Infarction Precipitated by a Pheochromocytoma in a Patient With Systemic Sclerosis

Abstract

Systemic sclerosis (SSc) patients typically experience Raynaud phenomena that is often complicated by digital ischemic lesions, gangrene, and digital loss. Other causes of peripheral ischemia, such as atherosclerosis, cryoglobulinemia, antiphospholipid syndrome, myeloproliferative disorders, paraneoplastic syndromes, and hyperadrenergic endocrine conditions, may be masked in SSc patients. We present a woman with limited SSc who developed toe necrosis and acute coronary events as a complication of a previously undiagnosed pheochromocytoma.

An unusual cause of toe necrosis

“…[1358–10] Significant negative correlation between plasma catecholamine concentration and skin blood flow has been demonstrated in pheochromocytoma. [1011] Resolution of ischemia and wound healing after removal of pheochromocytoma confirms the suggestion that excess catecholamine was responsible for the development of critical toe ischemia in our patient.…”

Phaeochromocytoma Mimicking Scleroderma

“…Phaeochromocytoma rarely presents with acute limb ischaemia [5, 6]. Significant negative correlations between plasma catecholamine concentration and dermal blood flow have been demonstrated in patients with phaeochromocytomas [7].…”

Phaeochromocytoma‐associated critical peripheral ischaemia

“…In adults, this phenomenon has only been described in 11 cases (Table 2). [3][4][5][6][7][8][9][10][11] The majority of patients reported were middle-aged women with a singular phaeochromocytoma with a diameter of about 5 cm. Although the relationships between tumour size, location and catecholamine levels are weak, 12 other evidence suggests a significant negative correlation between plasma catecholamine concentrations and skin blood flow.…”