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

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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.

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Authors and Affiliations

  1. Centre for Rheumatology, Royal Free and University College Medical School, University College, Rowland Hill Street, London, NW 32PF, UK

    Magdalena Dziadzio, Geraldine M. Brough, Carol M. Black & Christopher P. Denton

  2. Royal Free and University College Medical School, University College, London, UK

    Constantinos P. Anastassiades

  3. Department of Medicine, National Amyloidosis Centre, Royal Free Hospital, London, UK

    Philip N. Hawkins

  4. Department of Haematology, Royal Free Hospital, London, UK

    Michael Potter

  5. Istituto di Clinica Medica, Ematologia ed Immunologia Clinica, Università Politecnica delle Marche, Ancona, Italy

    Magdalena Dziadzio & Armando Gabrielli

Authors
  1. Magdalena Dziadzio
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  2. Constantinos P. Anastassiades
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  3. Philip N. Hawkins
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  8. Christopher P. Denton
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Correspondence to Christopher P. Denton.

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Dziadzio, M., Anastassiades, C.P., Hawkins, P.N. et al. From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature. Clin Rheumatol 25, 3–15 (2006). https://doi.org/10.1007/s10067-004-1076-3

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