Primary Systemic Amyloidosis

Abstract

Background:

Primary systemic amyloidosis is a rare disease with multiorgan involvement including the skin. Because of the nonspecific and variable presenting symptoms and signs, this condition usually follows a long course before the correct diagnosis is established. Fortunately, skin manifestations contribute greatly in revealing the disease and determining the amyloidosis type.

Objective:

This article reports a case of primary systemic amyloidosis mimicking nodular primary localized cutaneous amyloidosis of the legs. We describe our systemic findings and the investigation procedure to classify and characterize the amyloidosis type.

Methods and Results:

After histologic studies revealed amyloid substance in the skin nodules of our 82-year-old patient, she was hospitalized for an appropriate systemic evaluation of this condition and associated medical problems. The investigation included biochemical, immunologic, radiologic, pathologic, and histochemical methods.

Conclusion:

This case of primary systemic amyloidosis, simulating a localized cutaneous form with nodular lesions, demonstrates the importance of the distinction between both entities. In fact, the management and prognosis will vary significantly.