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Rapid Generation of Amyloid from Native Proteins In vitro
05:48

Rapid Generation of Amyloid from Native Proteins In vitro

Published on: December 5, 2013

[Primary systemic amyloidosis]

Srdan Tanasilović1, Dubravka Zivanović, Milos Nikolić

  • 1Klinicki centar Srbije, Institut za dermatovenerologiju, Beograd. drtanas@mail.com

Vojnosanitetski Pregled
|March 25, 2008
PubMed
Abstract

Insights

Systemic amyloidosis, a rare condition in older adults, presents with skin symptoms like "racoon sign" and macroglossia. Early diagnosis through histopathology is crucial for managing this severe disease.

Area of Science:

  • Dermatology
  • Hematology
  • Pathology

Background:

  • Systemic amyloidosis is a rare, often fatal disorder primarily affecting older individuals.
  • It can manifest as primary amyloidosis, sometimes linked to multiple myeloma, or secondary amyloidosis associated with other conditions.

Observation:

  • A 72-year-old male presented with distinctive skin lesions including periocular purpura ("racoon sign"), waxy papules, petechiae, and ecchymoses.
  • Additional symptoms included purpuric macules, macroglossia, significant weight loss, heart failure, and nephrotic syndrome.

Findings:

  • Immunoelectrophoresis confirmed circulating monoclonal protein immunoglobulin light chains.
  • Skin biopsy revealed Congo red-positive amyloid deposits in the dermis.
  • Cardiac and renal evaluations indicated advanced organ involvement.

Implications:

  • This case underscores the diagnostic significance of physical examination findings, such as "racoon sign" and macroglossia, in suspected systemic amyloidosis.
  • Prompt histopathological confirmation is vital for identifying amyloid deposition and guiding patient management.
  • Early recognition and diagnosis are critical for potentially improving outcomes in this rare and aggressive disease.

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