Melkerrson-Rosenthal syndrome wilth cardiac involvement

Abstract

Melkerrson-Rosenthal syndrome (MRS) is a granulomatous disease usually restricted to the orofacial region. We report a case of MRS in a 37-year-old Malay patient who presented with complete heart block on a background of recurrent oro-facial swelling and facial diplegia. Lip biopsy showed lymphohistocytic granulomatous inflammation typical for MRS. Extensive work-up excluded other causes of the complete heart block. To our knowledge, this is the first reported case of MRS affecting cardiac connective tissues and the first report of MRS in an ethnic Malay. We postulate granulomatous infiltration of the conductive tissues as the basis for the heart block. Another unusual feature of the case was the presence of left eye abduction limitation. © 2003 Elsevier Ltd. All rights reserved.