Primary systemic amyloidosis presenting as extensive cutaneous ulceration

Background: We report a case of primary systemic amyloidosis in a 78-year-old Caucasian woman presented as a nonhealing ulcer on the right thigh for 3 months. Histopathology of the skin revealed widely thickened walls of middermal and subcutaneous vessels from deposition of amorphous eosinophilic material that stained positively with Congo red and crystal violet. Objective: This case represents a very unusual presentation of primary systemic amyloidosis, one in which the cutaneous manifestations provided the first signs of a devastatingly widespread multiorgan infiltration of amyloid protein. Conclusion: This presentation of the disease may signify an advanced stage with a grave prognosis as our patient passed away 3 months after development of the cutaneous ulceration.