title: From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature.
abstract: Scleredema (also called scleredema of Buschke) is a fibromucinous connective tissue disorder of unknown cause that belongs to a group of scleroderma-like disorders. We report the case of a 64-year-old lady with long-standing scleredema, associated with a paraprotein, and progressing to multiple myeloma and AL amyloidosis. The relationship of scleredema with paraprotein and multiple myeloma is well established, but only two cases of scleredema associated with amyloidosis have been reported to date. We suggest that amyloidosis may be underdiagnosed in patients with scleredema and paraproteinaemia. Features attributed to extracutaneous manifestations of scleredema could represent systemic amyloidosis. We review published reports of scleredema associated with paraprotein and discuss the difficulties in the differential diagnosis of scleroderma-like disorders. We discuss the diagnosis of plasma cell dyscrasias and amyloidosis and their relevance in rheumatology practice.
authors.affiliation: Centre for Rheumatology, Royal Free and University College Medical School, University College, Rowland Hill Street, London, NW 32PF, UK.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Dziadzio
authors.forename: Constantinos P
authors.initials: CP
authors.lastname: Anastassiades
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.forename: Michael
authors.initials: M
authors.lastname: Potter
authors.forename: Armando
authors.initials: A
authors.lastname: Gabrielli
authors.forename: Geraldine M
authors.initials: GM
authors.lastname: Brough
authors.forename: Carol M
authors.initials: CM
authors.lastname: Black
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Denton
mesh_terms: D000686:Amyloidosis / Q000209:etiology* / Q000473:pathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D008875:Middle Aged; D009101:Multiple Myeloma / Q000209:etiology / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications / Q000473:pathology; D012592:Scleredema Adultorum / Q000150:complications* / Q000473:pathology
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