Background: Necrobiosis lipoidica (NL) is a member of the palisading granulomatous dermatitides that is associated, in most cases, with diabetes mellitus. However, there are an increasing number of cases of NL associa...Background: Necrobiosis lipoidica (NL) is a member of the palisading granulomatous dermatitides that is associated, in most cases, with diabetes mellitus. However, there are an increasing number of cases of NL associated with other forms of systemic disease. We describe a novel case of NL associated with a light- chain- restricted plasmacellular infiltrate; subsequent investigations established an underlying monoclonal gammopathy. Methods: Skin biopsy material was obtained and was processed in the usual fashion for hematoxylin and eosin (H& E) examination. Immunohistochemical staining was performed by utilizing kappa and lambda monoclonal antibodies (DakoCorporation,Carpentiera,CA,USA).Kappaandlambda in situ hybridization was also performed (Ventana Medical Systems, Tucson, AZ, USA). Results: A 55- year- old woman with a 5- year history of bilateral thigh subcutaneous nodules underwent a skin biopsy, showing typical changes of NL; there was a concomitant prominent perivascular plasmacellular infiltrate. Kappa light chain restriction was observed amid the plasmacellular infiltrate. Bone marrow biopsy and immunophenotyping studies revealed a clonal plasmacytosis with kappa light chain restriction. Conclusions: Granulomatous inflammation, including NL, may be a cutaneous paraneoplastic expression of low- grade B- cell lymphoproliferative disease in the context of an underlying plasma cell dyscrasia.展开更多
Cutaneous plasmacytosis is a rare disorder characterized by a benign proliferation of mature plasma cells that appears as multiple dark-brown to purplish skin lesions, often associated with polyclonal hypergammaglobul...Cutaneous plasmacytosis is a rare disorder characterized by a benign proliferation of mature plasma cells that appears as multiple dark-brown to purplish skin lesions, often associated with polyclonal hypergammaglobulinaemia. We present the case of a 55-year-old Caucasian man who suffered from a cutaneous plasmacytosis associated with two different carcinomas. Cutaneous plasmacytosis seems to be a reactive process because most cases reported are not associated with any apparent underlying disease. Nevertheless, because few reported cases were associated with malignancies, screening of additional neoplasms would be justified.展开更多
Background:Posttransplantation lymphoproliferative disorders (PTLDs) are lymphoid proliferations that can develop in recipients of solid organ or allogeneic bone marrow transplants. They are clinically and pathologica...Background:Posttransplantation lymphoproliferative disorders (PTLDs) are lymphoid proliferations that can develop in recipients of solid organ or allogeneic bone marrow transplants. They are clinically and pathologically heterogeneous and range from polyclonal hyperplastic lesions to malignant lymphomas. Although extranodal involvement in PTLD is common, cutaneous presentation is rare, with only 19 cases reported previously. Observations: We describe 4 patients with cutaneous presentations of PTLD. All patients had relatively lateonset PTLD (>1 year after transplantation) with a median of 8 years from organ allograft to tumor diagnosis. The extent, number, and anatomic location of skin lesions varied from a localized patch to widespread nodules. None of the patients exhibited systemic symptoms at the time of PTLD diagnosis. Pathological findings ranged from plasmacytic hyperplasia to monomorphic PTLD. In situ hybridization detected EpsteinBarr virus messenger RNA in all 3 cases with evaluable tissue. All patients underwent reduction in immunosuppressive therapy and received other individualized treatments. Median followup was 2.5 years. At the most recent followup, 3 patients were in complete remission and 1 had residual disease. Conclusions: In this study, PTLD lesions presenting in the skin responded to therapy. Despite their relatively late occurrence after transplantation, most of these cases were positive for EpsteinBarr virus. As observedwith other cutaneous lymphomas, the PTLDs with predominant skin involvement had a relatively favorable outcome.展开更多
文摘Background: Necrobiosis lipoidica (NL) is a member of the palisading granulomatous dermatitides that is associated, in most cases, with diabetes mellitus. However, there are an increasing number of cases of NL associated with other forms of systemic disease. We describe a novel case of NL associated with a light- chain- restricted plasmacellular infiltrate; subsequent investigations established an underlying monoclonal gammopathy. Methods: Skin biopsy material was obtained and was processed in the usual fashion for hematoxylin and eosin (H& E) examination. Immunohistochemical staining was performed by utilizing kappa and lambda monoclonal antibodies (DakoCorporation,Carpentiera,CA,USA).Kappaandlambda in situ hybridization was also performed (Ventana Medical Systems, Tucson, AZ, USA). Results: A 55- year- old woman with a 5- year history of bilateral thigh subcutaneous nodules underwent a skin biopsy, showing typical changes of NL; there was a concomitant prominent perivascular plasmacellular infiltrate. Kappa light chain restriction was observed amid the plasmacellular infiltrate. Bone marrow biopsy and immunophenotyping studies revealed a clonal plasmacytosis with kappa light chain restriction. Conclusions: Granulomatous inflammation, including NL, may be a cutaneous paraneoplastic expression of low- grade B- cell lymphoproliferative disease in the context of an underlying plasma cell dyscrasia.
文摘Cutaneous plasmacytosis is a rare disorder characterized by a benign proliferation of mature plasma cells that appears as multiple dark-brown to purplish skin lesions, often associated with polyclonal hypergammaglobulinaemia. We present the case of a 55-year-old Caucasian man who suffered from a cutaneous plasmacytosis associated with two different carcinomas. Cutaneous plasmacytosis seems to be a reactive process because most cases reported are not associated with any apparent underlying disease. Nevertheless, because few reported cases were associated with malignancies, screening of additional neoplasms would be justified.
文摘Background:Posttransplantation lymphoproliferative disorders (PTLDs) are lymphoid proliferations that can develop in recipients of solid organ or allogeneic bone marrow transplants. They are clinically and pathologically heterogeneous and range from polyclonal hyperplastic lesions to malignant lymphomas. Although extranodal involvement in PTLD is common, cutaneous presentation is rare, with only 19 cases reported previously. Observations: We describe 4 patients with cutaneous presentations of PTLD. All patients had relatively lateonset PTLD (>1 year after transplantation) with a median of 8 years from organ allograft to tumor diagnosis. The extent, number, and anatomic location of skin lesions varied from a localized patch to widespread nodules. None of the patients exhibited systemic symptoms at the time of PTLD diagnosis. Pathological findings ranged from plasmacytic hyperplasia to monomorphic PTLD. In situ hybridization detected EpsteinBarr virus messenger RNA in all 3 cases with evaluable tissue. All patients underwent reduction in immunosuppressive therapy and received other individualized treatments. Median followup was 2.5 years. At the most recent followup, 3 patients were in complete remission and 1 had residual disease. Conclusions: In this study, PTLD lesions presenting in the skin responded to therapy. Despite their relatively late occurrence after transplantation, most of these cases were positive for EpsteinBarr virus. As observedwith other cutaneous lymphomas, the PTLDs with predominant skin involvement had a relatively favorable outcome.