A Case of Primary Systemic Amyloidosis in a Patient with Multiple Myeloma.
- Author:
You In BAE
1
;
Sook Jung YUN
;
Seong Jin KIM
;
Seung Chul LEE
;
Young Ho WON
;
Jee Bum LEE
Author Information
1. Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea. jbmlee@chonnam.ac.kr
- Publication Type:Case Report
- Keywords:
Multiple myeloma;
Primary systemic amyloidosis
- MeSH:
Aged;
Amyloid;
Amyloidosis*;
Bacterial Infections;
Diagnosis;
Drug Therapy;
Electrophoresis;
Female;
Heart Failure;
Humans;
Immunoglobulins;
Macroglossia;
Multiple Myeloma*;
Paraproteinemias;
Peripheral Nervous System Diseases;
Purpura;
Renal Insufficiency;
Tongue
- From:Korean Journal of Dermatology
2007;45(12):1275-1279
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Primary systemic amyloidosis is associated with plasma cell dyscrasia, such as multiple myeloma. The amyloid fibrils in the disease are composed of amyloid light (AL) protein that is derived from immunoglobulin L chain. Cutaneous manifestations are presented in about 20~40% of patients with mainly petechia, purpura on flexural area of the body and waxy, translucent papulonodules. We report a case of primary systemic amyloidosis associated with multiple myeloma occurring in a 76-year old woman who had suffered from ecchymotic purpura on periorbital and flexural area with hemorrhagic bulla for one year. She also had macroglossia with grouped papulonodules on her tongue, and peripheral neuropathy. The final diagnosis was confirmed by positive PAS, Congo-red stain and specific serum/urine electrophoresis. She had been treated with several cycles of chemotherapy; however, she expired from various complications of the disease, such as congestive heart failure, renal failure, and secondary bacterial infection.
