1/49. Sezary cells with hairy projections.A case of sezary syndrome where the sezary cells showed cytoplasmic hairy projections is reported. The patient had typical exfoliative erythematous dermatitis, high white cell count, atypical lymphocytes of T-phenotype with folded nuclei and bone marrow involvement. The ultra structure study showed cerebriform nucleus and cytoplasmic projections.- - - - - - - - - - ranking = 1keywords = sezary syndrome, sezary (Clic here for more details about this article) |
2/49. sezary syndrome: a case report and a review of the molecular pathomechanism and management.sezary syndrome, one of the cutaneous T-cell lymphomas, is a rare cause of generalised exfoliative dermatitis. We report a case of sezary syndrome in a 64-year-old man who had persistent erythroderma for four years and who subsequently developed inguinal lymphadenopathy and marked leukocytosis. We review the pathomechanism and management of this rare condition.- - - - - - - - - - ranking = 0.0047988098193142keywords = erythroderma (Clic here for more details about this article) |
3/49. Combination treatment with extracorporeal photopheresis, interferon alfa and interleukin-2 in a patient with the sezary syndrome.Extracorporeal photopheresis is generally accepted as standard therapy for the leukaemic and erythrodermic variant of cutaneous T-cell lymphoma, the sezary syndrome (SS). Because of the limited efficacy in some patients with SS, combination therapy is often necessary. We report a new combination therapy for an intensively treated 62-year-old woman with advanced SS (T4N1BM1, stage IVb). Previous treatment with PUVA, retinoids alone and in combination with photopheresis, chlorambucil, and chemotherapy using cyclophosphamide, doxorubicin, vincristine and prednisone failed and were associated with significant side-effects. Six cycles of combination therapy with extracorporeal photopheresis, low-dose interferon alfa and interleukin-2 resulted in fading of the erythroderma and in a decrease of Sezary cells in the white blood cell count. The CD4/CD8 ratio decreased from 66 to 6 and the proportion of CD4 CD7 - cells from 47% to 11%. Only mild side-effects such as influenza-like symptoms, fever and nausea were observed. Two months after this therapy, the patient developed enlarged lymph nodes without erythroderma, and died 1 year later from the lymphoma. Combination therapy with extracorporeal photopheresis, interferon alfa and interleukin-2 might be useful in selected patients with SS.- - - - - - - - - - ranking = 0.0095976196386284keywords = erythroderma (Clic here for more details about this article) |
4/49. A case of pre-sezary syndrome preceded by hand lesions.Pre-sezary syndrome is an erythroderma with a chronic course, clinical findings of sezary syndrome, lymphocytic subepidermal band infiltration at times, and repeated cycles of circulating Sezary cells of less than 1,000 cells/mm3. Duration of the pre-existing skin diseases preceding pre-Sezary erythroderma varies from a few weeks to 20 years. Before the erythroderma develops, these patients are diagnosed with contact dermatitis, neurodermatitis, chronic dermatitis, atopic dermatitis, or asteatotic eczema. hand lesion also precedes the pre-Sezary erythroderma. This condition has been controlled by three cycles of chemotherapy consisting of vincristine, cytoxan, doxorubicin, and prednisolone. We describe a case of pre-sezary syndrome preceded by hand lesion and treated with chemotherapy.- - - - - - - - - - ranking = 0.019195239277257keywords = erythroderma (Clic here for more details about this article) |
5/49. Nonerythrodermic, leukemic variant of cutaneous T-cell lymphoma with indolent clinical course: Th2-type tumor cells lacking T-cell receptor/CD3 expression and coinfiltrating tumoricidal CD8 T cells.As typically represented by sezary syndrome, the leukemic form of cutaneous T-cell lymphoma (CTCL) mostly exhibits erythroderma. A patient with CTCL had slowly developing skin tumors as well as chronic leukemia. The tumor cell was CD4 CD7- th2 cells lacking T-cell receptor/CD3 complex and persistently occupied 27% to 48% of peripheral blood lymphocytes. In skin tumors, only 13% of tumor-infiltrating lymphocytes were malignant cells and substantial numbers of nonmalignant CD4 or CD8 T cells and B cells coinfiltrated. CD8 -infiltrating T cells had cytotoxic activity against the malignant T cell. Our case demonstrates the existence of the leukemic form of CTCL presenting with skin manifestation other than erythroderma and parapsoriatic patches. The nonerythrodermic feature and indolent course may be associated with the lack of T-cell receptor/CD3 expression and coinfiltration of a high percentage of nontumor lymphocytes, including tumoricidal CD8 T cells.- - - - - - - - - - ranking = 0.0095976196386284keywords = erythroderma (Clic here for more details about this article) |
6/49. Erythrodermic cutaneous T-cell lymphoma with disseminated pustulosis. Production of high levels of interleukin-8 by tumour cells.Interleukin (IL) -8 is a neutrophil chemoattractant cytokine with proinflammatory and growth-promoting activities, which is involved in the pathogenesis of several inflammatory diseases. It is found in high amounts in lesional biopsies of pustular diseases such as psoriasis and palmoplantar pustulosis. We report a 50-year-old woman with a 10-year history of erythroderma with disseminated pustulosis. Skin biopsies showed an epidermotropic infiltrate composed of atypical CD4 CD8 lymphocytes with numerous admixed neutrophils. Peripheral blood flow cytometric analysis revealed a major clonal subset of CD3 CD4 CD8 T-cell receptor Vbeta22 atypical lymphocytes. bone marrow biopsy, lymph node biopsy and computed thoracoabdominal tomography were normal. Serologies for human T-cell lymphotropic virus type I and human immunodeficiency virus were negative. Our patient's status deteriorated despite topical (nitrogen mustard, psoralen plus ultraviolet A) and systemic (interferon, methotrexate, multiagent chemotherapy) treatments, and she finally died. We showed that our patient's peripheral blood lymphocytes (PBL) spontaneously produced high amounts of IL-8. In contrast, PBL of patients with classical sezary syndrome produced lower amounts of IL-8. The production of IL-8 by tumour T cells could explain this unusual clinical and histopathological presentation of cutaneous T-cell lymphoma as disseminated pustulosis.- - - - - - - - - - ranking = 0.0047988098193142keywords = erythroderma (Clic here for more details about this article) |
7/49. Granulomatous slack skin: a distinct disorder or a variant of mycosis fungoides?About 75% of cutaneous lymphomas belong to the group of T-cell lymphomas. mycosis fungoides is the most common entity in this group. Granulomatous slack skin is a rare form of cutaneous T-cell lymphoma closely related to mycosis fungoides. We present here a patient with areas of lax skin for several years who developed a generalized erythroderma with associated immunoactivation and a deterioration in his general condition. This report discusses clinically and histologically the differential diagnoses, namely granulomatous slack skin and granulomatous mycosis fungoides, and suggests that these 2 disorders are only variants in the broad spectrum of a single disease.- - - - - - - - - - ranking = 0.0047988098193142keywords = erythroderma (Clic here for more details about this article) |
8/49. Transient CD30 nodal transformation of cutaneous T-cell lymphoma associated with cyclosporine treatment.BACKGROUND: mycosis fungoides (MF) may evolve from pre-existing chronic atopic or psoriasiform dermatitis and the histology can be equivocal. Early patch and plaque lesions of MF may evolve into tumors, disseminate to lymph nodes, bone marrow, and internal organs, and/or undergo transformation to a large cell size. methods: A patient with a history of "atopic dermatitis" followed by "psoriasis" rapidly developed exfoliative erythroderma and axillary lymphadenopathy following treatment with cyclosporine. At presentation, biopsy specimens of skin lesions and lymph nodes and staging were obtained. We present the treatment and follow-up of this patient and review the medical literature for similar cases. RESULTS: Multiple skin biopsy specimens from lesions revealed changes consistent with low-grade, cutaneous, T-cell lymphoma (MF) without evidence of large cell transformation and psoriasiform epidermal hyperplasia. CD30 large cell transformation was present in the lymph node. Adenopathy and erythroderma resolved without systemic therapy following discontinuation of cyclosporine and treatment with psoralen/ultraviolet A (PUVA), isotretinoin, interferon-alpha, and antimicrobials. CONCLUSIONS: This case documents a close relationship between atopy, psoriasis, and the development of cutaneous T-cell lymphoma, and illustrates that an immunosuppressive agent, cyclosporine, can dramatically alter the course of the disease.- - - - - - - - - - ranking = 0.0095976196386284keywords = erythroderma (Clic here for more details about this article) |
9/49. Bilateral breast involvement in sezary syndrome.Cutaneous T-cell lymphoma is a term used for mycosis fungoides and sezary syndrome, the distinct clinical entities where the skin is the primary organ of involvement. sezary syndrome is the leukemic variant of mycosis fungoides, presenting with generalized erythroderma, lymphadenopathy, and atypical cells (the Sezary cells) in the peripheral blood and bone marrow. The dissemination of cutaneous T-cell lymphoma may occur with no exception of the organs; however, no prior report exists about the sezary syndrome secondarily involving the breasts. We report the clinical and radiological findings of bilateral breast involvement in a case of sezary syndrome.- - - - - - - - - - ranking = 0.0047988098193142keywords = erythroderma (Clic here for more details about this article) |
10/49. Sustained remission of sezary syndrome.sezary syndrome, an aggressive form of cutaneous T-cell lymphoma, is a devastating, highly symptomatic form of non-Hodgkin lymphoma. Malignant clones of mature helper CD4 T cells containing large, convoluted nuclei known as Sezary cells circulate in the blood and infiltrate the skin. Clinical features include exfoliative erythroderma, generalized lymphadenopathy, alopecia, onychodystrophy, palmoplantar hyperkeratosis, and ectropion. patients often have severe pruritus, burning sensations, pain, bleeding from excoriations, and disfigurement. Extracorporeal photopheresis, an immunomodulatory therapy, has become a primary therapy for these patients. This pheresis-based therapy uses psoralen and ultraviolet A radiation-mediated photochemotherapy to induce immune responses. The effects of extracorporeal photopheresis vary considerably. We report sustained remission (2 years) in a patient with sezary syndrome. Previously he had received extracorporeal photopheresis and interferon alfa-2b injections. He is the only one of 55 patients with sezary syndrome treated at Mayo Clinic (Rochester, minnesota, USA) to achieve sustained remission on extracorporeal photopheresis alone.- - - - - - - - - - ranking = 0.0047988098193142keywords = erythroderma (Clic here for more details about this article) |
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