Abstract
Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain). Her serological workup including serum electrophoresis was negative and she underwent renal biopsy. Pathology revealed eosinophilic material within the mesangium that was Congo-red positive, had apple-green birefringence under polarized light, and ultramicroscopically appeared as fibrillary material. Subsequent bone marrow examination showed a diffuse increase in plasma cells with atypia indicating plasma cell neoplasm. This case underlines several interesting aspects of multiple myeloma and the way it may present with amyloidosis. The lack of monoclonal spike on electrophoresis yet positive light chain analysis deserves special attention by clinicians to avoid a missed diagnosis. The extensive skin involvement also raises several questions regarding the pathologic mechanisms of alopecia in a patient with amyloidosis.
Highlights
Amyloidosis results from the deposition of fibrils composed of low-molecular-weight subunits of a variety of proteins in the extracellular matrix
AL amyloidosis may result from the overproduction of immunoglobulin light chains, which can occur in monoclonal gammopathies, such as multiple myeloma (MM), as well as certain lymphoproliferative diseases.[7]
There is one reported case of alopecia universalis as the initial presentation of an occult amyloidosis
Summary
Amyloidosis results from the deposition of fibrils composed of low-molecular-weight subunits of a variety of proteins in the extracellular matrix. On review of the literature, we discovered 16 cases of amyloidosis with associated hair loss on examination.[13,14,15,16,17,18,19] There is one reported case of alopecia universalis as the initial presentation of an occult amyloidosis. Wheeler et al[14] described a case of a woman with anonychia, generalized hair loss, and weight loss for 18 months who was hospitalized with symptoms of heart failure and found to have amyloidosis associated with MM.
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callMore From: Journal of Investigative Medicine High Impact Case Reports
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
