Escleromixedema associado a miocardiopatia Scleromyxedema with associated cardiomyopathy

Relato de um caso de escleromixedema com lesões cutâneas associadas a miopatia, disfunção esofageana e paraproteinemia. Durante a evolução, a paciente apresentou sintomas de insuficiência cardíaca congestiva (ICC) que foram relacionados à miocardiopatia, passível de ocorrer em até 10% dos portadores de escleromixedema. São discutidos os aspectos clínicos, etiológicos e terapêuticos.We report a case of scleromyxedema with skin lesions in association with myopathy, esophageal dysfunction and paraproteinemia. During the disease course, the patient developed symptoms of cardiac failure related to a cardiomyopathy, a condition that has been described in up to 10% of patients with scleromyxedema. The clinical, etiologic and therapeutic aspects are discussed in this paper.