SAT-232 Pheochromocytoma Presenting Rare Skin Findings: Livedoid Vasculopathy and Raynaud’s Phenomenon

Abstract

BACKGROUND: Oversecreted catecholamines from pheochromocytoma (PCC) is responsible for a large variety of clinical signs and symptoms, including skin lesions, due to their effects on hemodynamics and metabolism, as well as the putative effects on the blood coagulation system. Livedo reticularis had been reported as the skin complications of PCC, but there was no pathological evidence. Livedoid vasculopathy, a subtype of livedo reticularis is a noninflammatory thrombotic condition. Here we firstly showed that livedo reticularis accompanying PCC.Case: A 36-year-old female with hypertension, hyperglycemia, and right adrenal mass had referred to our hospital. She had violaceous reticular lesions on bilateral lower limbs, a three-year history of recurrent ulcers around her right ankle with severe pain. She also had frequent Raynaud’s phenomenon. On admission, the blood examination showed that her hemoglobin concentration was 16.3g/dl and her platelet count was 408,000/ml. Plasma catecholamines and the metabolite levels were elevated. The abdominal CT scan showed the right adrenal mass, approximately 6.5cm in diameter, and MRI showed a heterogeneous mass with high signal intensity on T2-weighted images. MIBG scintigraphy showed uptake in the mass. We diagnosed her as PCC. A skin biopsy revealed occlusions of the lumen of small vessels in the mid dermis without vasculitis. Administration of doxazosin revealed the improvement of the clinical parameters including the pain and the ulcer of the right ankle. The laparoscopic right adrenalectomy was performed. Histopathological study of the adrenal tumor was consistent with PCC. Genetic testing and MLPA analysis for RET, VHL, SDHx, SDH-AF2, MAX, and TMEM127 showed no mutations or copy number alterations. Postoperatively, hypertension and hyperglycemia improved, and Raynaud’s phenomenon and reticular macules disappeared.Discussion: Among the various clinical signs of PCC, skin disorders are relatively rare. Limited reports showed that the livedo reticularis was a complication of PCC, but its mechanism had not been elucidated. Livedoid vasculopathy is a rare cutaneous disease manifesting as recurrent ulcers on the lower extremities, which is a noninflammatory thrombotic condition associated with hypercoagulation. The catecholamine excess could induce arterial vasospasm in addition to relative hypercoagulation tendency, which could result in livedoid vasculopathy.Conclusion: The present study showed first pathological evidence of that the livedo reticularis accompanying PCC was livedoid vasculopathy.