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Amyloid and amyloidosis.

S M Breathnach1

  • 1Department of Medicine (Dermatology), Charing Cross and Westminster Medical School, London, U.K.

Journal of the American Academy of Dermatology
|January 1, 1988
PubMed
Summary
This summary is machine-generated.

Cutaneous lesions, often waxy or purpuric, can indicate underlying plasma cell dyscrasia in systemic amyloidosis. Early recognition of these skin manifestations is key for diagnosis and management.

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Area of Science:

  • Dermatology
  • Hematology
  • Pathology

Background:

  • Cutaneous lesions affect up to 40% of patients with systemic amyloidosis.
  • These lesions result from immunoglobulin light chain deposition, often linked to plasma cell dyscrasias.
  • Mucocutaneous lesions, carpal tunnel syndrome, and macroglossia are characteristic indicators.

Purpose of the Study:

  • To review the clinical presentation and significance of cutaneous lesions in various forms of amyloidosis.
  • To discuss the diagnostic implications of skin manifestations in plasma cell dyscrasias.
  • To summarize current understanding of localized and systemic cutaneous amyloidosis.

Main Methods:

  • Review of existing literature on cutaneous manifestations of amyloidosis.
  • Analysis of clinical features associated with primary, myeloma-associated, and secondary systemic amyloidosis.

Related Experiment Videos

  • Examination of localized cutaneous amyloidosis variants, including nodular, macular, and papular forms.

    • Main Results:

    • Waxy, purpuric mucocutaneous lesions are crucial early indicators of occult plasma cell dyscrasia.
    • Localized cutaneous amyloidosis lesions can mimic systemic forms but arise from local plasma cell infiltration.
    • Macular amyloid and lichen amyloidosus often present with chronic pruritus and may have familial or racial predilections.

    Conclusions:

    • Cutaneous lesions are significant diagnostic markers for systemic and localized amyloidosis.
    • Prognosis for systemic amyloidosis related to plasma cell dyscrasia remains poor despite limited treatment responses.
    • Management strategies vary, with colchicine for familial Mediterranean fever-associated amyloidosis and potential roles for DMSO and retinoids in specific cases.