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[Bullous amyloidosis].

J L Schmutz1, A Barbaud, J F Cuny

  • 1Clinique Dermatologique de Nancy, Hôpital Fournier.

Annales De Dermatologie Et De Venereologie
|January 1, 1988
PubMed
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This study details a rare case of a 75-year-old man with extensive skin lesions, including epidermal separation and ecchymotic patches, diagnosed as non-myelomatous lambda-2 monoclonal gammopathy with amyloidosis.

Area of Science:

  • Dermatology
  • Nephrology
  • Hematology

Background:

  • Primary amyloidosis is a rare systemic disease characterized by extracellular deposition of misfolded proteins.
  • Bullous lesions are an uncommon manifestation of primary amyloidosis, posing diagnostic challenges.

Observation:

  • A 75-year-old male presented with widespread skin lesions including epidermal separation, ecchymotic patches, and hemorrhagic erosions.
  • Physical examination revealed a positive Nikolsky's sign and the presence of epidermal cysts.
  • Histopathology demonstrated eosinophilic deposits and intradermal bullae with amyloid-like fibrillar structures.

Findings:

  • Direct immunofluorescence showed absence of immunoglobulins and complement components.
  • Laboratory tests revealed lambda-2 monoclonal gammopathy with Bence-Jones protein and 8% plasmocytes in bone marrow.

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  • Diagnosis confirmed as non-myelomatous lambda-2 monoclonal dysglobulinaemia with systemic amyloid deposits.

    • Implications:

    • This case highlights the importance of considering monoclonal gammopathies and amyloidosis in patients presenting with unusual bullous dermatoses.
    • Early diagnosis and management of underlying monoclonal gammopathy are crucial for patient outcomes.
    • Further research into the pathogenesis of cutaneous manifestations in amyloidosis is warranted.