1/8. The Shulman syndrome.The Shulman syndrome is a symptom complex recently described in the rheumatology literature that is characterized by eosinophilia, hypergammaglobulinemia, and a diffuse scleroderma-like process of the extremities. The onset of illness has been associated with a period of unusual physical exertion. The condition seems to respond to corticosteroid therapy for systemic effect.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/8. Eosinophilic fasciitis.Shulman's syndrome includes fasciitis of rapid onset, sclerodermoid skin changes, absence of Raynaud's phenomenon, absence of visceral manifestations of progressive systemic sclerosis, hypergammaglobulinemia, transient eosinophilia, and a dramatic response to systemic corticosteroid therapy. Although this syndrome may be a variant of scleroderma, its dramatic responsiveness to corticosteroid therapy is a particularly distinguishing feature.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/8. Idiopathic portal hypertension associated with progressive systemic sclerosis.Herein we describe the clinical course of a case of idiopathic portal hypertension accompanied by splenomegaly, portal hypertension, and pancytopenia in addition to progressive systemic sclerosis. Immunological studies revealed positive antinuclear antibodies, hypergammaglobulinemia with increases in IgG and IgA, a decrease in peripheral T lymphocytes, and a decrease in the rate of lymphocyte blast transformation induced with phytohemagglutinin. The spleen weighed 1070 g, and the portal pressure was 270 nm H2O. Esophageal varices were also noted. These findings suggest the possibility of association of some immunological abnormalities with the etiology of idiopathic portal hypertension.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/8. Coexistence of rheumatoid arthritis and systemic sclerosis in four patients.The classic skin and internal organ system manifestations of systemic sclerosis developed in 4 individuals, each with an erosive, destructive chronic polyarthritis characteristic of rheumatoid arthritis. All had serum rheumatoid factors, anti-nuclear antibodies, hypergammaglobulinemia and circulating immune complexes. Each of the 4 has been resistant to treatment. The features of these patients, together with an analysis of this unusual coexistence of two connective tissue disorders, are presented.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/8. Diffuse fasciitis with eosinophilia.The recently described syndrome of diffuse fasciitis with eosinophilia consists of scleroderma-like cutaneous changes in the absence of Raynaud's phenomenon and without visceral involvement. Peripheral blood eosinophilia and hypergammaglobulinemia occur in the majority of patients. The biopsy specimen is characteristic and shows a diffusely thickened fascia with a noticeable inflammatory infiltrate. The etiology and pathogenesis of the entity are unknown. The majority of the cases have been reported to respond to corticosteroids. Pulmonary function abnormalities and rheumatoid factor elevation in one of our patients suggests that this syndrome may be a variant scleroderma or may have previously unrecognized systemic manifestations. The patients' objective responses to corticosteroids were minimal.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
6/8. A new case of eosinophilic fasciitis.A new case of Shulman's disease is reported. The patient shows skin induration in both arms and legs, and morphea plaques without visceral involvement. Onset of the disease followed an unusual physical effort. An important blood eosinophilia and a mild hypergammaglobulinemia are present. The histopathological studies show a typical eosinophilic fasciitis. The use of steroids had no effect in the treatment of the disease.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/8. 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) |
8/8. Eosinophilic fasciitis.A detailed evaluation of the clinical and histologic manifestations of eosinophilic fasciitis is described in a 22-year-old woman. Scleroderma-like skin involvement is associated with eosinophilia and hypergammaglobulinemia. Visceral and serologic manifestations of a generalized scleroderma are lacking. Results of a biopsy showed severe thickening of the deep fascia accompanied by an infiltration of lymphocytes and plasma cells. This syndrome must be differentiated from generalized scleroderma because eosinophilic fascitis has a benign course and may benefit from systemic corticosteroid therapy.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |