Erosive arthropathy associated with scleromyxedema

Abstract

Mucin deposition in the skin (scleromyxedema) is a rare disorder with a historically confusing nomenclature. The etiology and pathogenesis of the disease is poorly understood. The precise extent of potential systemic involvement is uncertain but the prevailing hypothesis is that scleromyxedema is a disorder of skin fibroblasts without internal organ involvement. Two patients with scleromyxedema skin lesions are described in whom an erosive arthropathy developed in joints contiguous to clinically involved skin. Because of the absence of other causes of destructive arthritis, it is postulated that the associated skin disease resulted in infiltration of adjacent synovium with subsequent erosions.