Coexistence of papular mucinosis and systemic amyloidosis associated with lambda-type IgD paraproteinemia.
The patient is an obese, 55-year-old woman. She noticed purpura at several sites when she was 49 years old. She visited our clinic with a chief complaint of exertional dyspnea at 51 years of age. Physical examination revealed localized edema in the left chest wall and lower abdomen with translucent papules in the center. Macroglossia, hemorrhagic macules, loss of axillary and pubic hairs, and goose-egg sized swellings of submaxillary lymph nodes were also found. Laboratory data were within the normal range except an increase of IgD and low ECG voltage. Bone-marrow puncture revealed an increase (27%) of plasma cells with some atypicality. Serum immunoelectrophoresis clarified IgD lambda-type paraproteinemia and lambda-type Bence Jones proteinemia. Histologically, edema with an increase in GAG was conspicuous in the circumscribed areas of the middle dermis. Amyloid deposition was clarified in the walls of arterioles in the deep dermis.
Ishibashi
A
A
Nakabayashi
K
K
Kukita
A
A
D000686:Amyloidosis / Q000150:complications* / Q000188:drug therapy; D004487:Edema / Q000473:pathology; D005260:Female; D006801:Humans; D007072:Immunoglobulin D / Q000032:analysis*; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D008875:Middle Aged; D009077:Mucins / Q000032:analysis; D010265:Paraproteinemias / Q000150:complications*; D012871:Skin Diseases / Q000150:complications* / Q000473:pathology; D013194:Staining and Labeling
D007072:Immunoglobulin D; D007147:Immunoglobulin Light Chains; D009077:Mucins