Response of hemorrhagic bullous skin lesions of the breast secondary to primary systemic amyloidosis to a five-drug combination chemotherapy: a case report and review of the literature

Primary and secondary amyloidosis are the two major types of amyloidosis. The primary systemic amyloidosis, also known as light chain (AL) amyloidosis, is mostly related to a plasma cell dyscrasia. The secondary (AA) amyloidosis is derived from serum amyloid A subunit protein, an acute-phase protein that is produced in response to inflammatory conditions [1]. There is no identifiable, underlying cause of AL amyloidosis [2]. The fibrils of AL amyloidosis are composed of polymerized immunoglobulin light chain or light chain fragments [3]. Amyloid protein is resistant to proteolysis and has a three dimensional configuration as a beta pleated sheet [4]. Although cutaneous involvement in primary systemic amyloidosis is relatively common [5], the occurrence of bullous skin lesions is rare [2,3].Only a few cases of cutaneous involvement with systemic light chain amyloidosis have been reported in the literature. Skin involvement in the form of hemorrhagic bullous is much rarer. To the best of our knowledge, hemorrhagic bullous presentation of amyloidosis on the breast skin has not been reported in the literature. This uncommon presentation of amyloidosis was only partially responsive to the commonly used combination chemotherapy regimens, but it responded completely to a five-drug combination regimen. This suggests that a more aggressive approach, with combination of multiple immunomodulators and chemotherapy agents may be required to achieve a meaningful response in similar cases.A 51-year-old African American female with no significant past medical history presented in April 2009 with a 1-month history of hemorrhagic skin lesions on both breasts. The patient had no other symptoms. She was not taking any medication, and her social and family histories were noncontributory. Physical examination revealed extensive bullous ulcerating and hemorrhagic skin lesions involving posterior aspects of both breasts and upper abdominal skin bilaterally (Figure 1). An initial diagnostic