Abstract
We report a case of papular cutaneous amyloidosis associated with multiple myeloma in a 52 years old man who presented papular xanthoma like lesions in periorbital areas and scrotum with severe bone pain in thoracic and lumbar spinal column and lower limbs. Cutaneous histopathology revealed the presence of amorphous deposits in papillary dermis surrounded with small lymphoid infiltrates with erythrocyte containing slots. Congo red staining was positive and bone marrow aspiration showed dysplastic plasma cells. Lytic lesions were observed in long bones and skull radiography. 24 hours Proteinuria was 3.69 g / l. A combination of Melphalan and prednisone as treatment could restore renal function without any change in skin lesions after 14 months.
Highlights
Amyloid is defined as the deposition of insoluble protein fibrils, forming histologically a homogeneous, eosinophilic mass, which stains positive for the Congo red dye and displays green birefringence under polarized light because of its β-pleated sheet conformation [1]
We report a case of a cutaneous papular amyloidosis associated with multiple myeloma
The sequential analysis of amino acids in systemic amyloidosis associated with multiple myeloma has shown that the amyloid fibrils are composed of an AL protein, which in turn are composed of variable region of immunoglobulin light chain, intact light chain, or both [6]
Summary
Amyloid is defined as the deposition of insoluble protein fibrils, forming histologically a homogeneous, eosinophilic mass, which stains positive for the Congo red dye and displays green birefringence under polarized light because of its β-pleated sheet conformation [1]. Immunoglobulin light chain–derived (AL) amyloidosis with an estimated 500 new cases per year in France is the most frequent amyloid disorder in the Western world [3]. It is usually caused by indolent plasma cell clone which synthesizes an unstable, misfolded monoclonal immunoglobulin light chain that is prone to aggregate and form amyloid fibrils. Cutaneous amyloidosis is characterized by deposition of amyloid in skin, which can be isolated or in association with systemic amyloidosis. Cutaneous manifestations of amyloidosis may occasionally be the initial presentation of systemic amyloidosis. We report a case of a cutaneous papular amyloidosis associated with multiple myeloma
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 callDisclaimer: 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.
