1/26. common variable immunodeficiency and eosinophilic fasciitis.The association of eosinophilic fasciitis and immunological defects is rare, especially hypogammaglobulinemia. We report a case of eosinophilic fasciitis occurring in a female, 53 years old, with common variable immunodeficiency. The diagnosis of common variable immunodeficiency was established by chance observation of persistently low levels of all immunoglobulin classes unrelated to protein loss or immunosuppressive treatment, one year after the appearance of eosinophilic fasciitis, which is usually characterized by hypergammaglobulinemia. Our description may prompt the investigation of an increased rate of simultaneous occurrence of eosinophilic fasciitis and common variable immunodeficiency.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/26. Eosinophilic fasciitis associated with simple traumatism.Eosinophilic fasciiitis is an idiopathic and uncommon condition, a scleroderma-like disorder that predominantly affects the extremities and is characterized by marked thickening and inflammation of the fascia, associated with with peripheral blood eosinophilia, hypergammaglobulinemia, and characteristic histologic findings. A case is presented of a 75-year-old female patient who sought medical assistance for sudden occurrence of induration and erythema of both lower extremities one week after she had fallen from a one-meter height, with generalization of the signs. The diagnosis of eosinophilic fasciitis was made on the basis of histopathologic findings, and treatment with glucocorticoids, non-steroid anti-inflammatory drugs and antihistaminics was prescribed.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/26. Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis.Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.- - - - - - - - - - ranking = 7.2917715104516keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
4/26. Recurrent eosinophilic cystitis with peripheral eosinophilia and hyperimmunoglobulinemia E.A case of recurrent eosinophilic cystitis with peripheral eosinophilia and hyperimmunoglobulinemia E, which responded successfully to initial and secondary steroid therapies is reported.- - - - - - - - - - ranking = 9.1147143880645keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
5/26. Inflammatory pseudotumor of the liver and peripheral eosinophilia in autoimmune pancreatitis.AIM: Inflammatory pseudotumor (IPT) of the liver is a rare benign lesion, the etiology of which remains obscure. It is not associated with any particular diseases apart from phlebitis and Crohn's disease. methods: A middle-aged male with hepatic IPT and peripheral eosinophilia associated with autoimmune pancreatitis (AIP) was selected for this study and review of literature. RESULTS: A 59-year-old male was admitted with obstructive jaundice, marked eosinophilia (1 343/mm(3)) and hypergammaglobulinemia (4 145 mg/dL). Imaging techniques revealed dilatation of the intrahepatic bile duct, stenosis of the common bile duct with diffuse wall thickening, gallbladder wall thickening, irregular narrowing of the pancreatic duct, and swelling of the pancreatic parenchyma. Multiple liver masses were also demonstrated and diagnosed as IPT by biopsy specimens. Six months later, the abnormal features of the biliary tree remarkably improved by the oral administration of prednisolone, and the liver masses disappeared. The swelling of the pancreatic head also improved. The peripheral eosinophil count normalized. IPT associated with AIP, as we know, has not been reported in the literature. The clinical features of the present case mimicked those of pancreatic cancer with liver metastasis. This case deserves to be documented to prevent misdiagnosis of similar cases.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/26. Eosinophilic fasciitis associated with autoimmune thyroiditis.Eosinophilic fasciitis (EF) is scleroderma-like disease without Raynaud's phenomenon or visceral involvement. It is characterized by painful swelling of the extremities, accompanied by rapid weight gain, fever and myalgia. The acute state of disease is associated with significant peripheral blood eosinophilia, an elevated erythrocyte sedimentation rate and hypergammaglobulinemia. EF is also frequently associated with hematological abnormalities, including malignant lymphoproliferative diseases, but rarely associated with autoimmune thyroiditis. In the present study we report a case of eosinophilic fasciitis associated with autoimmune thyroiditis.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/26. Eosinophilic fasciitis in a child mimicking a myopathy.A 14-year-old boy was suspected of having a myopathy with joint contractures. He presented with progressive painless joint contractures of his right wrist and fingers, and reduced muscle strength of his right arm, without obvious skin changes. Laboratory investigation showed a normal CK, hypergammaglobulinemia and eosinophilia. ultrasonography revealed thickened fasciae in the forearm. A full thickness biopsy confirmed the diagnosis of eosinophilic fasciitis. The contractures diminished quickly with prednisolone and methotrexate. The ultrasound scans 2 years after diagnosis showed improvement, although some thickening of the fasciae was still present, indicating residual changes. In conclusion, eosinophilic fasciitis has to be regarded as a differential diagnosis of painless joint contractures in children. ultrasonography can be helpful to suspect the diagnosis.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/26. A syndrome characterized by nodular eosinophilic infiltration of the skin and immunoglobulin isotype imbalance.A 9-year-old boy had recurrent acute, plaquelike, nodular infiltrations with overlying vesicles and bullae of the face and right hand. Histologically, the lesions consisted of a perivascular and periadnexal lymphohistiocytic infiltrate with many eosinophils. The lesions responded to dapsone therapy. Laboratory studies revealed blood eosinophilia, hyperimmunoglobulinemias E and G4, and hypoimmunoglobulinemias M and G1-3, which normalized after treatment. The patient's immune deviation is consistent with a transient imbalance of lymphokine production in helper T cells.- - - - - - - - - - ranking = 1.8229428776129keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
9/26. Variant of hyper-IgE syndrome: the differentiation from atopic dermatitis is important because of treatment and prognosis.The hyper-IgE syndrome is characterized clinically by recurrent staphylococcal abscesses of the skin, lungs and other sites from infancy. Affected patients also have a pruritic dermatitis that differs in character and distribution from lesions of atopic dermatitis. Most lack other signs of atopic disease, develop persistent pneumatoceles and have osteopenia. Laboratory abnormalities include the consistent presence of marked hyperimmunoglobulinemia E and eosinophilia of blood, sputum and tissues. They may have other inconsistent abnormalities of humoral and cellular immune responses and sometimes of phagocytic cell chemotactic responsiveness. Other clinical problems reported in such patients have included lymphomas, cryptococcal meningitis and cutaneous fungal disease. An 18-year-old male patient with a variant of the hyper-IgE syndrome, which he had acquired after a measles attack at the age of 5 years, suffered from recurrent ulcerative dermatitis and lymph node abscesses. Immunological investigation revealed an excessively elevated total serum IgE level (46,850 IU/ml), the presence of specific IgE to staphylococci, and quantitative and functional deficiency of IgG2. skin and serological (radioallergosorbent) tests to inhalant and nutritive allergens were negative. Differentiation from atopic dermatitis should be made, because a long-term antistaphylococcal regime not only improves skin lesions but hinders the occurrence of lung abscesses and pneumatoceles.- - - - - - - - - - ranking = 1.8229428776129keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
10/26. Eosinophilic fasciitis during pregnancy.The first reported case of eosinophilic fasciitis developing in pregnancy is discussed, and the obstetric management is reported. Eosinophilic fasciitis is a rare disease characterized by pain, swelling, and tenderness over the extremities, followed by induration of the skin. Laboratory findings include peripheral eosinophilia, hypergammaglobulinemia, an elevated erythrocyte sedimentation rate, distinctive histopathologic changes, and scleroderma-like skin induration without rheumatoid serologic markers. diagnosis is made by biopsy of the deep fascia of the affected area. The deep fascia will be infiltrated with plasma cells, lymphocytes, and eosinophils. prednisone is the therapy of choice. carpal tunnel syndrome and rare serious hematologic abnormalities have been associated with eosinophilic fasciitis.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
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