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Case for diagnosis. Systemic light chain amyloidosis with cutaneous involvement
João Renato Vianna Gontijo1, Jackson Machado Pinto1, Maysa Carla de Paula1
1Dermatology Clinic - Santa Casa de Belo Horizonte - Belo Horizonte (MG), Brazil.
Anais Brasileiros De Dermatologia
|November 23, 2017
Summary
Systemic light chain amyloidosis, a rare disease, often presents with skin lesions. Early diagnosis by dermatologists is crucial, as exemplified by a case with eye and cardiac involvement.
Area of Science:
- Hematology
- Dermatology
- Cardiology
Background:
- Systemic light chain amyloidosis (AL) is a rare plasma cell dyscrasia.
- Cutaneous manifestations are common and crucial for early diagnosis by dermatologists.
- Clinical presentation is diverse, often unspecific, and depends on the affected organ.
Observation:
- A case of palpebral amyloidosis with bilateral ecchymoses and cardiac involvement is presented.
- The patient had no evidence of plasma cell dyscrasia or lymphoma.
- The diagnosis was confirmed via biopsy.
Findings:
- Systemic light chain amyloidosis can present with prominent cutaneous and cardiac findings.
- Diagnosis requires a high index of suspicion and biopsy confirmation.
- The condition can progress rapidly, even without overt plasma cell malignancy.
Implications:
- Highlights the critical role of dermatologists in diagnosing systemic light chain amyloidosis.
- Emphasizes the importance of recognizing cutaneous signs for early detection.
- Underscores the potential for rapid deterioration and poor prognosis in systemic amyloidosis.
