Oligosecretory Myeloma With Amyloidosis and Alopecia

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.

The role of trichoscopy beyond hair and scalp diseases: A review

“…49 Skin involvement is observed in 29%-40% of all cases of systemic amyloidosis. 50 Alopecia in patients with systemic amyloidosis is rarely observed and presents as patchy or diffuse nonscarring progressive hair thinning. 34,51 There is only one case report about trichoscopic features of systemic amyloidosis.…”

“…Systemic amyloidosis is an uncommon disorder in which misfolded protein becomes resistant to the body's catabolic processes and fibrils deposit extracellularly within tissues 49 . Skin involvement is observed in 29%–40% of all cases of systemic amyloidosis 50 . Alopecia in patients with systemic amyloidosis is rarely observed and presents as patchy or diffuse nonscarring progressive hair thinning 34,51 .…”

Classic features of primary systemic amyloidosis (AL amyloidosis) leading to diagnosis of plasma cell myeloma

“…Dermatology Online Journal || Photovignette lesions, cutis laxa, scalp lesions resembling cutis verticis gyrata, nail dystrophies, alopecia, and scleroderma-like infiltrative plaques [6][7][8][9][10][11][12]. Patients with AL amyloidosis present with a wide range of associated symptoms, including weight loss, fatigue, syncope, peripheral edema, peripheral neuropathy, carpal tunnel syndrome, and cutaneous symptoms.…”