Clinical Rheumatology

, Volume 25, Issue 1, pp 3–15

From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature

  • Magdalena Dziadzio
  • Constantinos P. Anastassiades
  • Philip N. Hawkins
  • Michael Potter
  • Armando Gabrielli
  • Geraldine M. Brough
  • Carol M. Black
  • Christopher P. Denton
Review

DOI: 10.1007/s10067-004-1076-3

Cite this article as:
Dziadzio, M., Anastassiades, C.P., Hawkins, P.N. et al. Clin Rheumatol (2006) 25: 3. doi:10.1007/s10067-004-1076-3

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.

Keywords

AmyloidosisMultiple myelomaParaproteinScleredema adultorum

Copyright information

© Clinical Rheumatology 2005

Authors and Affiliations

  • Magdalena Dziadzio
    • 1
    • 5
  • Constantinos P. Anastassiades
    • 2
  • Philip N. Hawkins
    • 3
  • Michael Potter
    • 4
  • Armando Gabrielli
    • 5
  • Geraldine M. Brough
    • 1
  • Carol M. Black
    • 1
  • Christopher P. Denton
    • 1
  1. 1.Centre for Rheumatology, Royal Free and University College Medical SchoolUniversity CollegeLondonUK
  2. 2.Royal Free and University College Medical SchoolUniversity CollegeLondonUK
  3. 3.Department of Medicine, National Amyloidosis CentreRoyal Free HospitalLondonUK
  4. 4.Department of HaematologyRoyal Free HospitalLondonUK
  5. 5.Istituto di Clinica Medica, Ematologia ed Immunologia ClinicaUniversità Politecnica delle MarcheAnconaItaly