Gammopathy-associated acrosclerosis as a cause of flexion contractures in an older adult patient: a case report and literature review.

摘要

This article describes an 87-year-old woman with progressive debilitating flexion contractures involving both hands. In addition, a similar scariform process in this patient extended to involve the upper and lower extremities. The patient had an established history of an JgG monoclonal gammopathy of undetermined significance (MGUS). Her contraction deformities were treated surgically. This is the first known reported case of gammopathy-associated acrosclerosis eventuating in bilateral hand contractures. IgG lambda paraprotein may evoke fibrocyte hypercellularity and sclerosis. Recent studies have used chemotherapy and peripheral stem cell transplantation in the treatment of severe sclerosing reactions associated with MGUS. This more recent approach reflects the inherent basis of the fibrosis, at least in scleromyxedema, and is one that is reflective of a clonal B-cell lymphoproliferative disorder.