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Updated: Jun 20, 2026
12:23
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
[Purpura: primary systemic amyloidosis manifestation]
Sara Lestre1, Andreia Gonçalves, Alexandre João
1Hospital Santo António dos Capuchos, Lisboa.
Acta Medica Portuguesa
|August 19, 2009
Summary
Primary Systemic Amyloidosis (AL) is a frequent condition where immunoglobulin light chains deposit in organs. Mucocutaneous signs like purpura are key early diagnostic clues for this amyloidosis.
Area of Science:
- Hematology
- Dermatology
- Pathology
Background:
- Primary Systemic Amyloidosis (AL) is the most common systemic amyloidosis, characterized by immunoglobulin light chain deposition in organs.
- Mucocutaneous manifestations occur in 30-40% of AL cases and are crucial for early diagnostic suspicion.
Observation:
- A 71-year-old female presented with disseminated purpura and cutaneous fragility for 6 months, plus recent dysphagia.
- Initial lab evaluations were normal. Skin and subcutaneous fat biopsies revealed lambda type amyloid protein deposition.
- Systemic work-up showed proteinuria (>1g/24h), Bence Jones proteins, and monoclonal immunoglobulin light chain (lambda type) in serum.
Findings:
- Histological and immunohistochemical analysis confirmed lambda type amyloid protein.
- Diagnostic confirmation of primary systemic amyloidosis was established through laboratory and biopsy findings.
Implications:
- Early recognition of mucocutaneous signs can aid in the timely diagnosis of AL amyloidosis.
- This case highlights the importance of a comprehensive diagnostic approach, integrating clinical, histological, and laboratory findings.
- Treatment with prednisolone and melphalan was initiated upon diagnosis.
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