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

Cutaneous and systemic plasmacytosis (CSP) is a rare disorder of unknown etiology characterized by cutaneous polyclonal plasma cell infiltrates associated with various extracutaneous involvement and polyclonal hypergammaglobulinemia. Here, we report on a 54-year-old male patient with chronic renal insufficiency who presented with disseminated reddish-brown macules and plaques on the face and trunk. In our evaluation, he was found to have lymphadenopathy, polyclonal hypergammaglobulinemia; benign plasma cell infiltration involving the skin, bone marrow, and retroperitoneal area; and renal amyloidosis. To the best of our knowledge, this is the first reported case of CSP associated with renal amyloidosis.
Amyloidosis* ; Bone Marrow ; Humans ; Hypergammaglobulinemia ; Lymphatic Diseases ; Male ; Middle Aged ; Plasma Cells ; Renal Insufficiency, Chronic ; Skin

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

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

The development of malignant tumors is a more frequent finding in lupus patients than the general population. However, the incidence of multiple myeloma associated with systemic lupus erythematosus (SLE) is a rare manifestation. SLE is an autoimmune disease characterized by B cell hyperactivity, formation of autoantibodies, and multi-clonal hypergammaglobulinemia. Serum immunoglobulin increases, with a specific increase in IgG, which is due to an increase in autoantibody formation related to extrinsic and intrinsic antigens. We report on a patient with SLE combined with IgG, lambda type nonsecretory multiple myeloma, which was diagnosed with refractory anemia and hypergammaglobulinemia.
Anemia, Refractory ; Autoantibodies ; Autoimmune Diseases ; Humans ; Hypergammaglobulinemia ; Immunoglobulin G ; Immunoglobulins ; Incidence ; Lupus Erythematosus, Systemic ; Multiple Myeloma

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

We report two patients with multiple peculiar skin eruptions and polyclonal hypergammaglobulinemia. Both patients visited our hospital for the evaluation of asymptomatic multiple nodular eruptions on almost their entire body except for the lower extremities. Histologic examinations disclosed prominent infiltration of plasma cells and lymphoid follicular hyperplasia in the dermis but these plasma cells showed neither a mitotic figure nor atypicalities. Laboratory examinations showed polyclonal hypergammaglobulinemias and increased erythrocyte sedimentation rates. In spite of various investigations, the cause of the hypergammaglobulinemia remained obscure.
Blood Sedimentation ; Dermis ; Humans ; Hypergammaglobulinemia* ; Hyperplasia ; Lower Extremity ; Plasma Cells ; Skin

We reviewed one case of AHA in 14 year old female child which followed a chronic course. The patient showed hypergammaglobulinemia with elevated IgM, positive direct coomb's test and positive cold aggutinin test. The patient improved with steroid therapy, and over the past six months has showen a fair course. We have presented a review of the literature briefly and given our report.
Adolescent ; Anemia, Hemolytic, Autoimmune* ; Child ; Coombs Test ; Female ; Humans ; Hypergammaglobulinemia ; Immunoglobulin M

OBJECTIVE: To evaluate the clinical features, laboratory and imaging results, treatment and outcomes of eosinophilic fasciitis (EF) and assess the value of ultrasound in the diagnosis of EF. METHODS: We retrospectively analyzed the clinical data of 45 patients with EF treated in our center from January 1, 2006 to February 28, 2022. The consistency between the diagnoses of EF based on ultrasound and MRI findings was assessed. RESULTS: In the 45 EF patients (male/female ratio 3.5:1), the age of onset ranged from 16 to 64 years with a mean disease course of 22.6 months. The average time from symptom onset to diagnosis was 16 months. The most common possible trigger of the disease was vigorous exercise (10/45), causing symmetrical lesions in the limbs, most commonly in the forearms (86.7%) and lower legs (80%). Clinical features of EF included subcutaneous swelling and induration (95.6%), arthralgia and arthritis (55.6%), groove sign (42.2%), hand joint contractures (42.2%), skin pigmentation (37.8%), and peau d'orange appearance (13.3%). Eosinophilia was found in 31 patients (68.9%). Hypergammaglobulinemia was seen in 23/44 (52.3%) and positive antinuclear antibodies in 9 (20%) of the patients. Twentyone of the patients were treated with high-dose methylprednisolone (≥200 mg daily for 3 to 5 consecutive days), and compared with the patients who did not receive this treatment, these patients more frequently experienced relapse before admission, had more extensive involvement, and had a higher rate of hypergammaglobulinemia without fever, but these differences were not statistically significant. Of the 31 patients (68.9%) with follow-up data (for a median of 3.2 years [range 0.2-15.9]), complete remission was achieved in 12 (38.7%) patients, and the accumulative complete remission rate was 44.1% at 5.5 years. No specific baseline characteristics or immunosuppressants were found to correlate with the treatment response. A total of 26 patients underwent both ultrasound and MRI examination, and the Kappa value of the diagnostic results between ultrasound and MRI was 0.91. CONCLUSION EF is characterized by symmetrical subcutaneous swelling and induration in the limbs, accompanied by eosinophilia and hypergammaglobulinemia. Glucocorticoid is effective for treating EF. Ultrasound examination can identify thickening of subcutaneous fascia for an early diagnosis of EF.
Humans ; Female ; Male ; Infant ; Child, Preschool ; Retrospective Studies ; Hypergammaglobulinemia ; Eosinophilia ; Ultrasonography ; Hand ; Contracture ; Treatment Outcome

Hypergammaglobulinemic purpura of Waldenstrom is characterized by recurrent episodes of petechiae, hypergammaglobulinemia, an elevated erythrocyte sedimentation rate, and the presence of rheumatoid factor, and leukocytoclastic vasculitis or mild lymphocytic perivasculitis. There is a primary type and a secondary type which is associated with other autoimmune diseases. We present a csae of a 48 year-old female with hypergammaglobulinemic purpura of Waldenstrom. She showed characteristic clinical, histopathological and laboratory findings consistent with hypergammaglobulinema purpura of Waldenstrom. Although there was a decrease in the Schimer test, we couldt den ostrate an association with Sjogrens syndrome. We think that this case may be a secondary type and needs a follow-up study.
Autoimmune Diseases ; Blood Sedimentation ; Female ; Follow-Up Studies ; Humans ; Hypergammaglobulinemia ; Middle Aged ; Purpura ; Purpura, Hyperglobulinemic* ; Rheumatoid Factor ; Sjogren's Syndrome ; Vasculitis