Scleromyxedema Presenting With Neurologic Symptoms: A Case Report and Review of the Literature
This article has been peer reviewed and approved by Michael Fisher, MD, Professor of Medicine, Albert Einstein College of Medicine. Review date: February 2010.
Drs. Marshall, Klepeiss, and Helm report no conflict of interest. Dr. Ioffreda is a consultant for Johnson & Johnson. This relationship is not relevant to this article. The authors report no discussion of off-label use. Dr. Fisher reports no conflict of interest. The staff of CCME of Albert Einstein College of Medicine and Cutis® have no conflicts of interest with commercial interest related directly or indirectly to this educational activity. From the Penn State Milton S. Hershey Medical Center and Penn State College of Medicine, Hershey. Dr. Marshall is a plastic surgery resident and Dr. Klepeiss was a resident, the Department of Dermatology. Dr. Ioffreda is Associate Professor and Dr. Helm is Professor, both from the Departments of Dermatology and Pathology. Dr. Klepeiss currently is a dermatologist, Geisinger Health System, State College, Pennsylvania.
Kathleen Marshall, MD; Stacy A. Klepeiss, MD; Michael D. Ioffreda, MD; Klaus F. Helm, MD
Scleromyxedema is a rare variant of lichen myxedematosus. In addition to cutaneous manifestations, scleromyxedema often presents with systemic manifestations, including dysphagia, proximal muscle weakness, central nervous system disturbances, encephalopathy, and restrictive lung disease. It is almost always associated with paraproteinemia, usually IgG with γ light chains. We review the literature on scleromyxedema associated with neurologic symptoms and present a case of a 49-year-old woman with encephalopathy attributable to scleromyxedema.