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A3474 - Cardiac Scleromyxedema Causing Cardiogenic Shock and Death
Author Block: M. C. Runnstrom1, H. Li1, G. Graves2, D. C. Patel2, A. Ataya2; 1Department of Medicine, University of Florida, Gainesville, FL, United States, 2Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, University of Florida, Gainesville, FL, United States.
Introduction:
Scleromyxedema is a rare idiopathic disorder characterized by cutaneous eruptions composed of mucin deposits, fibroblast proliferation and commonly monoclonal gammopathy. This disorder is distinct from that seen with connective tissue deposits seen in myxedema from thyroid disease. Systemic involvement can involve most organs. The disease is chronic and no treatment has been validated to induce long lasting remission.
Case report:
A 68-year-old female with a history of hypothyroidism, atrial fibrillation and known scleromyxedema, presented with acute progressive dyspnea requiring intubation and mechanical ventilation and hypotension requiring vasopressors.
Contrasted computed tomography (CT) of the chest revealed moderate sized bilateral pleural effusions and compressive atelectasis but no pulmonary embolism. Blood cultures did not grow any organisms.
Over the next two days, she became oliguric and AST and ALT increased from normal to 5341 and 3550, respectively. The patient was persistently hypotensive despite being on maximum doses of four vasopressors. Bedside transthoracic echocardiography showed elevated right ventricular pressures and a new decrease in left ventricular ejection fraction from recently being normal. Nebulized epoprostenol was started but despite these interventions, the patient passed away from cardiogenic shock.
Postmortem examination showed extensive eosinophilic deposition within the interstitium of the myocardium, kidneys and dermis. This eosinophilic deposition was Alcian blue positive and Congo red negative, consistent with mucin. Exam further revealed cardiac biventricular hypertrophy and dilation of bilateral atria, as well as congestive hepatopathy with centrilobular necrosis. The lungs were heavy with diffuse alveolar damage and the main pulmonary arteries were without thrombus.
Discussion:
Scleromyxedema, sometimes considered an aggressive form of papular mucinosis, is a rare idiopathic chronic mucin deposition disorder. It usually appears in middle-aged men and women and has a poor overall prognosis. Although no treatment has been reliably validated, intravenous immunoglobulin and corticosteroids have induced temporary lack of progression of the disease. The optimal treatment is unknown. Bos et al published a review of 17 cases of Autologous Stem Cell Transplantation as the ultimate treatment for scleromyxedema, with 59 % achieving initial remission, but at a median follow up of >40 months, only 12 % were still disease free.
Our patient likely succumbed secondary to her multi-organ failure as a result of cardiogenic shock from her mucin deposition disease, demonstrated by the extensive mucin deposition found on postmortem examination. A rare manifestation of this disease.