Complications of Scleredema Adultorum

\s=b\Multiple myeloma and congestive heart failure developed in a patient with long-standing scleredema adultorum. Staining of the myocardium, performed after her death, was positive for acid mucopolysaccharide and negative for amyloid. To the best of our knowledge, this is the first case in which acid mucopolysaccharide has been demonstrated in the myocardium, thus explaining the cardiomyopathy of scleredema adultorum. Review ofthe world literature enabled us to identify a statistically significant increased prevalence of plasma cell dyscrasia among patients with protracted scleredema. In all patients, plasma cell dyscrasia appeared years after the onset of scleredema. Immunofluorescent studies were negative for immunoglobulin deposition. We assume, therefore, that the plasma cell dyscrasia was secondary to scleredema. (Arch Intern Med 1988;148:551-553) Ocleredema adultorum ie a rare connective tiesue disorder ^-J of unknown etiology. It is characterized by a firm, nonpitting edema, which typically begins at the neck and epreade gradually to involve the face, ecalp, ehoulders, and trunk. Skin biopsiee reveal marked thickening of the dermie due to collagenoue replacement of the subcutis and depoeition of hyaluronic acid between the collagen fibers. Hyaluronic acid, a noneulfated acid mucopolyeaccharide, etaine with alcian blue (pH, 2.5 to 4.0), colloidal iron, and toluidine blue (pH, 4.0 to 7.0). Prior digestion with hyaluronidase negates the reaction. There are three types of scleredema1: type 1, the claesic dieeaee, ae described by Buechke, with preceding febrile illneee and eventual resolution in a period of monthe to two yeare; type 2, a etatic or elowly progreeeive dieeaee over many yeare, with no preceding febrile illness; and type 3, diabetic scleredema, a nonreeolving, elowly progressive scleredema in diabetic patients. Systemic involvement associated with nondiabetic scleredema ie uncommon. Next to skin, the jointe, heart, tongue, eyee, and ekeletal muecles are moet frequently affected. Plasma cell dyecraeia has been reported occasionally.28