No abstract available.
Cryoglobulinemia* ; Gangrene* ; Monoclonal Gammopathy of Undetermined Significance* ; Paraproteinemias*

No abstract available.
Monoclonal Gammopathy of Undetermined Significance* ; Paraproteinemias* ; Vasculitis

Cutaneous and systemic plasmacytosis is a rare disorder that's characterized by a cutaneous plasma cell infiltrate accompanied by polyclonal hypergammaglobulinemia. Clinically, it appears as multiple red-brown plaques that mainly occur on the trunk, and this is sometimes associated with variable extracutaneous involvement. All the cases of cutaneous and systemic plasmacytosis reported in Korea have shown the typical skin manifestations on the trunk of the patients. Herein, we report on a case of cutaneous and systemic plasmacytosis and the patient presented with multiple erythematous to brownish patches on the face, neck and both axillary areas.
Humans ; Hypergammaglobulinemia ; Korea ; Neck ; Plasma Cells ; Skin Manifestations

Renal diseases with organized deposits include amyloid, fibrillary, immunotactoid, and cryoglobulinemic glomerulopathies. AL amyloidosis and fibrillary glomerulonephritis are different in the composition of their immunoglobulin deposits. Fibrils of fibrillary glomerulonephritis are usually composed of polyclonal, occasionally oligoclonal or monoclonal, immunoglobin G, but amyloidosis consists of monoclonal light chains. Simultaneous occurrence of fibrillary glomerulonephritis and AL amyloidosis is very rare. We report a case of fibrillary glomerulonephritis combined with AL amyloidosis in a 71-yr-old man.
Amyloid ; Amyloidosis ; Glomerulonephritis ; Immunoglobulins ; Light ; Monoclonal Gammopathy of Undetermined Significance ; Paraproteinemias

This review focuses on the clinical use of (18)F-FDG PET to evaluate multiple myeloma. (18)F-FDG PET is useful for diagnosis, staging of multiple myeloma and differential diagnosis of myeloma related disease such as monoclonal gammopathy of undetermined significance or plasmacytoma. For therapy response, (18)F-FDG PET may be effective after chemotherapy for multiple myeloma and radiotherapy for plasmacytoma.
Diagnosis, Differential ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Plasmacytoma

Humans ; Paraproteinemias ; Monoclonal Gammopathy of Undetermined Significance ; Kidney ; Mass Spectrometry

Anemia ; Humans ; Male ; Middle Aged ; Monoclonal Gammopathy of Undetermined Significance ; Paraproteinemias

Eosinophilic faciitis is a recently recognized entity causing inflammation, thickening and fibrosis of the fascia, which is associated with hypergammaglobulinemia, peripheral eosinolhila. This case of 11 year-old male patient who complaint migrating arthralgia and flexion contration with nodule on left upper extremity for 6 month and suddenly developed proptosis had hypergammaglobulinemia and elevated ESR. Biopsy of the nodule demonstrated inflammatory infiltration of eosinophil in fascia and subcutis, which was consisted with eoainophilic fasciitis. We report a case of eosinophilic fasciitis with brief review who was treated with corticosteroid and had symptomatic improvement.
Arthralgia ; Biopsy ; Child ; Eosinophils* ; Exophthalmos ; Fascia ; Fasciitis* ; Fibrosis ; Humans ; Hypergammaglobulinemia ; Inflammation ; Male ; Upper Extremity

Autoimmune hepatitis is defined as an unresolving, predominantly periportal hepatitis of unknown etiology, usually with hypergammaglobulinemia and tissue autoantibodies, which is responsive to immunosuppressive therapy. Clinical manifestations, diagnostic criteria, differential diagnosis and treatment guideline have been described. In Korea the prevalence of autoimmune hepatitis seemed to be lower than that in western countries, but clinical features were similar, except for more severe female preponderance. According to recent clinical survey, mean age is 47.8, and MF ratio is 9:1. Mostly type 1, and had cirrhosis in 22% at the time of diagnosis. 16% of patients had associated immunologic disorders. The diagnosis is definite is 47.3%, and probable in 51.3%. 72% were treated with immunosuppressive therapy with remission rate of 70%. To understand the clinical features of autoimmune hepatitis adquately, nationalwide prospective clinical and epidemiological studies are needed urgently.
Autoantibodies ; Diagnosis ; Diagnosis, Differential ; Female ; Fibrosis ; Hepatitis ; Hepatitis, Autoimmune* ; Humans ; Hypergammaglobulinemia ; Korea ; Prevalence

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is a benign, idiopathic histiocytic proliferative disorder affecting lymph nodes as well as extranodal sites. It is accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. Although cutaneous involvement in RDD is common, a purely cutaneous disease is very rare. Histologic findings show characteristic large, pale, histiocytic cells exhibiting cytophagocytosis (emperipolesis). Immunohistochemically, these histiocytes are positive for S-100 protein and CD68, but negative for CD1a. The etiology is unknown, although it is thought to be a reactive disorder rather than neoplastic. We report two cases of Rosai-Dorfman disease showing involvement limited to the skin.
Adjustment Disorders ; Blood Sedimentation ; Cytophagocytosis ; Fever ; Histiocytes ; Histiocytosis, Sinus* ; Hypergammaglobulinemia ; Leukocytosis ; Lymph Nodes ; S100 Proteins ; Skin