1/10. Cutaneous Rosai-Dorfman disease.Presented is a case of a 31-year-old woman with cutaneous Rosai-Dorfman disease, which is a rare, benign, generally self-limited histiocytic proliferative disorder that is characterized by painless lymphadenopathy, fever, neutrophilia, an elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. It primarily involves lymph nodes but can affect extranodal sites such as the skin, where it typically appears as indistinct erythematous papules and nodules. On histopathologic examination, emperipolesis is a consistent finding. The etiology is unknown, and treatment is based on clinical manifestations.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
2/10. Cutaneous plasmacytosis: A report of five cases with immunohistochemical evaluation for HHV-8 expression.Cutaneous plasmacytosis is a rare disorder that typically affects middle-aged to older individuals of Asian, particularly Japanese, descent. Clinically, it is characterized by multiple asymptomatic red-brown plaques and nodules on the trunk. Lymphadenopathy and hypergammaglobulinemia may be present. Histologically, the lesions show a moderately dense superficial and deep perivascular infiltrate composed predominantly of mature plasma cells without atypia or light chain restriction. We report our experience with five additional cases, including results of immunohistochemical studies for human herpes virus 8.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
3/10. Primary anetoderma associated with a wide spectrum of autoimmune abnormalities.Although the underlying pathologic mechanisms of primary anetoderma have not yet been identified, data suggest the participation of an immunologic mechanism in some cases. In a woman with clinical and histopathologic features of primary anetoderma (Jadassohn-Pellizzari type) of 30 years' duration, laboratory investigation disclosed positive antinuclear factor, hypocomplementemia, hypergammaglobulinemia, granular deposits of immunoreactants along the dermoepidermal junction, and fibrillar deposits in the papillary dermis. In addition, she was found to have autoimmune hemolysis and circulating lupus anticoagulant associated with recurrent deep-vein thrombosis and a history of Graves' disease (starting 5 years after onset of primary anetoderma). To our knowledge, none of the latter three autoimmune conditions has been previously associated with primary anetoderma.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
4/10. Systemic plasmacytosis. A syndrome of peculiar multiple skin eruptions, generalized lymphadenopathy, and polyclonal hypergammaglobulinemia.A description is given of two patients with peculiar multiple skin eruptions, asymptomatic generalized lymphadenopathy, and polyclonal hypergammaglobulinemia. Both patients were admitted to our hospital for further evaluation of an increased erythrocyte sedimentation rate and hypergammaglobulinemia discovered during routine medical examinations. Despite various investigations, the underlying disease causing the hypergammaglobulinemia was not found. Histologic examination disclosed dense perivascular infiltration of plasma cells in the dermis. In the lymph nodes, considerable plasma cell infiltration was found from the cortex to the medulla. These plasma cells were mature and showed no cellular atypism. The association of peculiar multiple skin eruptions, lymphadenopathy, and polyclonal hypergammaglobulinemia, which we have called "systemic plasmacytosis," signifies a new syndrome that can be differentiated from diseases reported previously.- - - - - - - - - - ranking = 8keywords = hypergammaglobulinemia (Clic here for more details about this article) |
5/10. 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) |
6/10. Systemic plasmacytosis: a case which improved with melphalan.Plasmacytosis, a distinctive proliferative disorder of plasma cells, is characterized by peculiar multiple skin eruptions, lymphadenopathy and polyclonal hypergammaglobulinemia. To date there has been no report of such cases showing remarkable responses to therapeutic agents. We herein report a case of plasmacytosis which developed in a 52-year-old Korean man and showed remarkable improvement with melphalan.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
7/10. Primary cutaneous plasmacytosis: report of three cases and review of the literature.BACKGROUND: Cutaneous plasmacytosis is a rare disease characterized by peculiar multiple eruptions and hypergammaglobulinemia. More than 40 cases have been reported, mainly in japan, although information concerning the disorder was limited to individual case reports. OBJECTIVE AND methods: To clarify the clinicopathological and laboratory features, we reviewed 41 cases. RESULTS: All patients were Japanese and the male-to-female ratio was 1:0.6. The onset ages ranged from 20 to 62 years, with a mean and median of 37 and 37 years. A superficial lymphadenopathy was detected in 58% (22/38), and polyclonal hypergammaglobulinemia was found in 93% (38/41). No cases were associated with any apparent underlying diseases. The course was chronic without spontaneous remission. Four patients died, 3 of whom succumbed to leukemia, respiratory failure or renal failure, respectively. CONCLUSION: The results suggest that the condition appears to be a variant of reactive plasmacytic disorders of unknown origin.- - - - - - - - - - ranking = 2keywords = hypergammaglobulinemia (Clic here for more details about this article) |
8/10. Cutaneous plasmacytosis: report of a case in a white man.We describe a 40-year-old white man with a peculiar skin eruption in association with polyclonal hypergammaglobulinemia. No underlying disease was detected. A skin biopsy specimen showed a proliferation of mature plasma cells intermingled with some lymphocytes and histiocytes, an appearance consistent with cutaneous plasmacytosis. This disease had been previously described only in Japanese patients. In our patient the disease progressed slowly. Lymph node infiltration by mature plasma cells was later noted.- - - - - - - - - - ranking = 1keywords = hypergammaglobulinemia (Clic here for more details about this article) |
9/10. 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) |
10/10. Case of malignant lymphoma associated with primary systemic plasmacytosis with polyclonal hypergammaglobulinemia.Systemic plasmacytosis (SP), which has a histologic appearance similar to that of multicentric Castleman's disease (MCD), is also known as benign plasma cell proliferation with polyclonal hypergammaglobulinemia, cutaneous plasmacytosis, and/or generalized plasmacytic lymphadenopathy. The prognosis of SP reportedly has been good. A 59-year-old Japanese man was treated for multiple cutaneous lesions of his trunk as well as polyclonal hypergammaglobulinemia. A skin biopsy showed infiltration of lymphocytes and polyclonal plasma cells in the dermis. The patient developed enlarged superficial lymph nodes 5 years later, and T-cell lymphoma, diffuse mixed type, was diagnosed. At that time, his cutaneous plasmacytosis remained but the polyclonal hypergammaglobulinemia had resolved. Ours is the first reported case of SP to be complicated by the development of T-cell lymphoma.- - - - - - - - - - ranking = 7keywords = hypergammaglobulinemia (Clic here for more details about this article) |
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