1/11. 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) |
2/11. 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) |
3/11. 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) |
4/11. 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) |
5/11. 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) |
6/11. Eosinophilic fasciitis presenting as inflammatory polyarthritis.Eosinophilic fasciitis is an uncommon disorder characterized by peripheral blood eosinophilia, hypergammaglobulinemia, and scleroderma-like inflammation of skin and fascia. Joint contractures occur but typically without synovitis and effusion. Our patient presented with symmetric polyarthritis and subsequently characteristic features of eosinophilic fasciitis developed. It is important to consider this disorder in patients presenting with arthritis as well as those presenting with skin changes because prior treatment with corticosteroids can alter biopsy findings, leading to diagnostic confusion, and possible adverse effects on the long-term treatment regimen.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/11. Eosinophilic fasciitis: a distinct clinical entity?Eosinophilic fasciitis is a syndrome characterized by exertion related scleroderma-like skin changes, peripheral eosinophilia, hypergammaglobulinemia and diffuse faciitis. Controversy exists as to the precise classification of the syndrome, i.e., whether it is a distinct entity or a variant of scleroderma. We describe a patient with eosinophilic faciitis but with several unique features: 1) progressive skin changes unresponsive to corticosteroid therapy; 2) elevated anti-dna antibodies; 3) hypocomplementemia; and 4) a followup biopsy showing sclerodermatoid skin changes. These features and others relating to the controversial aspects of classification of eosinophilic fasciitis are discussed.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/11. Eosinophilic fasciitis: report of a case with features of connective tissue diseases.A patient suffering from eosinophilic facsiitis is described and the literature reviewed. The original description of eosinophilic fasciitis was that of a benign scleroderma-like disease with eosinophilia, hypergammaglobulinemia and diffuse thickening and inflammation of the fascia and subcutaneous tissue. Our patient like a few others, presented additional findings, namely, Raynaud's phenomenon and mild myhositis. She is the first patient described with sjogren's syndrome. It is important that eosinophilic faciitis be recognized clinically because it seems to be a benign and treatable entity.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/11. Eosinophilic fasciitis in an adolescent girl with lymphadenopathy and vitiligo-like and linear scleroderma-like changes. A case report.The patient, a 14-year-old girl, suffered from arthralgias which occurred after tonsillitis. Two months later she developed edema of the left lower extremity, finger flexion contractures and induration of the skin of the left leg, associated with hypergammaglobulinemia, peripheral hypereosinophilia, elevated ESR and a positivity of ANA and anti ds-dna antibodies. A biopsy of the inguinal lymph node, performed because of left inguinal and retroperitoneal lymphadenopathy, showed only slight inflammatory activation and a granulomatous reaction after lymphography. A few days after the lymphography linear erythema evolving later into hyperpigmentation and corresponding to the superficial lymphatics developed on the left side of the body, very probably as a reaction to the patent-blue dye. Deep en-block skin biopsy confirmed the diagnosis of eosinophilic fasciitis (EF). After two years of therapy with prednisone and d-penicillamine the patient felt well, and her flexion contractures resolved, ANA were positive, while anti ds-dna were negative. Linear hyperpigmentation persisted, and linear scleroderma-like changes developed on the left lower limb. A vitiligo-like lesion on the right foot which occurred after one year of therapy persisted. The possible risk of developing systemic connective tissue disease necessitates the long term follow up of this patient.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
10/11. polymerase chain reaction of borrelia burgdorferi flagellin gene in Shulman syndrome.A 49-year-old man presented a progressive swelling and induration of the skin resulting in flexion contracture. He had a history of two tick bites at the age of 17 and 47 years. serum anti-Borrelia-burgdorferi antibody was positive; isolation of B. burgdorferi from the skin lesion was unsuccessful. He had eosinophilia (white blood cells 8,300/microlitre, 33% eosinophils) and hypergammaglobulinemia. The diagnosis of Shulman syndrome (eosinophilic fasciitis) from clinical and histological findings was established. A part of the flagellin gene of B. burgdorferi was detected in a skin biopsy sample by using the polymerase chain reaction method. To the best of our knowledge, this is the first report of detection of B.-burgdorferi-specific dna from a skin sample of Shulman syndrome.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
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