BACKGROUND: Intravascular lymphoma is a rare disease characterized by the proliferation of neoplastic monuclear cells within the lumens of small blood vessels. The neoplastic cells are usually of B-cell origin, and rarely of T-cell or histiocytic origin. Although this clinicopathological entity of lymphoma has not been listed in general pathological classifications such as REAL classification or the Working Formulation, it is recently in the WHO classification scheme, which is essentially an updated REAL scheme, and the EORTC classification scheme. METHODS: In this report, a 62-year-old woman with intravascular large B-cell lymphoma was observed by clinical, histopathological, immunohistochemical and molecular methods. RESULTS: A 62-year-old woman presented with large erythematous macules on the bilateral thighs and lower legs. The lesions were accompanied with hard, tender, intradermal or subcutaneous nodules mimicking erythema nodosum. Histopathological examination in the first biopsy revealed non-specific panniculitis compatible with erythema nodosum. The second biopsy revealed emboli of atypical lymphocytes within many of the dilated and proliferated vessels in the deep dermis and subcutaneous tissue. These cells were positive for L-26 and kappa light chain, and negative for lambda light chain, factor VIII-related antigen, CD30, CD34, CD68 and UCHL-1. These findings confirmed the diagnosis of intravascular large B-cell lymphoma. A laboratory examination showed a high level of LDH and abnormal cells in the bone marrow. An MRI of the brain and computed tomographic (CT) scans of the chest and abdomen revealed no evidence of malignancy. Before the treatment, the size of the nodules decreased spontaneously by about 50% in one month and significantly in two months. Although combination chemotherapy, which consisted of CHOP, brought her partial remission, she experienced neurological symptoms 6 months after the initial treatment and died of brain metastasis 9 months after the treatment. CONCLUSIONS: This is a unique case for two following reasons: 1) the first biopsy revealed non-specific findings compatible with erythema nodosum; and 2) before the treatment, the nodules regressed spontaneously. Dermatologists should take multiple skin biopsies for EN lesions with the non-specific histopathological findings not to refute the existence of this disease. 相似文献
BACKGROUND: During the course of immunodeficiency diseases, severe candidiasis can occur with extensive cutaneous and mucous membrane lesions. However, blood dyscrasias are very rarely revealed by diffuse candidiasis. We report two case of cutaneous T-cell lymphoma revealed by extensive and atypical cutaneous candidiasis. PATIENTS AND METHODS: Case No. 1:A 72-year-old woman presented a pruritic rash of circinate, serpiginous patches on glabrous skin and skinfolds with multiple intertrigo and rapidly worsening palmoplantar keratoderma. All mycological skin specimens tested positive for Candida albicans. Histological examination of a biopsy sample from a serpiginous patch revealed the presence of fungal elements while palmoplantar keratoderma biopsy showed an epidermotropic lymphocytic infiltrate in the superficial dermis evocative of mycosis fungoides. Blood tests showed a white cell count of 28 600/mm3 with 14% circulating Sezary cells and a T-cell clone. The T-cell lymphoma was treated with methotrexate, but the disease worsened a few months later, progressing to CD30- large T-cell pleomorphic lymphoma. The patient died of severe sepsis. Case No 2:A 60-year-old man presented a macular rash over the face, trunk and skinfolds as well as erythematous scaly annular plaques of the glabrous skin with lymphadenopathy. Cultures of skin scrapings were all positive for Candida albicans. Blood tests showed a white cell count of 15 000/mm3 with 30% circulating Sezary cells. A trunk patch biopsy revealed the histological appearance of mycosis fungoides. There was a T-cell clone in the peripheral blood and skin. DISCUSSION: In both cases, the patients presented with widespread annular and erythematous scaly lesions of the glabrous skin and skinfolds with evidence of Candida albicans on fungal tests of all skin scrapings. The discovery of circulating Sezary cells on a systematic smear for hyperleukocytosis led us to suspect underlying cutaneous T-cell lymphoma, which was confirmed by biopsy of the skin lesions accompanying the mycoses. Widespread cutaneous candidiasis can occur in patients with cell-mediated immunodepression. Cutaneous T-cell lymphoma can enhance such candidiasis through interference with skin integrity and impairment of cell-mediated immunity, with large amounts of IL10 and TGF-B, increased secretion of soluble interleukin-2 receptors (CD25) and impaired CD8 suppressor cell function. 相似文献
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 Sézary 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. 相似文献
A 32-year-old white man with no previous medical past was admitted to our department with an 8-month intermittent history of acute spiking fever with axillary, inguinal, and neck lymphadenopathy, arthritis, and cutaneous eruption. Cutaneous examination showed nonpruriginous, indurated erythematous papules affecting the face, ears, and trunk, suggestive of subacute lupus erythematosus ( Fig. 1a,b ). On general physical examination, no abnormalities were observed except for arthritis of the right ankle and, a few months later, arthritis of the left knee. Biological investigations revealed a raised erythrocyte sedimentation rate with a serum ferritin level at 800 ng/mL (normal < 200 ng/mL), a white blood cell count of 12,500/mm3 (with 80% neutrophils, 16% lymphocytes, and 4% monocytes), a hematocrit of 40%, and a hemoglobin level of 13.1 g/dL. Electrolytes and liver function tests were normal. Antinuclear antibodies and antibodies to deoxyribonucleic acid (DNA) were negative. Rheumatoid factor was absent. No evidence of the following infections/organisms was found on serologic screening: syphilis, Streptococcus, Toxoplasma, Mycoplasma, Brucella, Borrelia burgdorgferi, spotted fever, leptospirosis, hepatitis B and C virus, parvovirus, human immunodeficiency virus (HIV) 1 and 2, human T-cell lymphotropic virus (HTLV) 1, Epstein–Barr virus (EBV), cytomegalovirus (CMV), herpes simplex virus (HSV) 6. Immunoglobulin M (IgM) HSV(1+2) by enzyme-linked immunosorbent assay (ELISA) analysis with 1 year follow-up was still detected. The articular synovial fluid of the involved ankle and knee was inflammatory but sterile. A bone marrow biopsy specimen revealed no lymphomatous involvement. Abdominal and chest scans were normal. Skin biopsy of a facial nodule showed, in the dermis and in the subcutaneous fat tissue, a dense perivascular and periadnexal infiltrate composed of numerous plasmacytoid histiocytes and large- or medium-sized lymphocytes ( Fig. 2a,b ). No epidermal changes, edema in the papillary dermis, or vasculitis were found. Immunofluorescence staining of frozen tissue sections of a facial nodule showed granular deposits of C3. Inguinal lymph node biopsy revealed, in the paracortical area, focal necrosis with a proliferation of transformed lymphocytes, histiocytes, and numerous nuclear debris typical of histiocytic necrotizing lymphadenitis ( Fig. 3a,b ). On immunohistochemical staining examination, CD68 was positive in pathologic paracortical areas. Few mononuclear cells were positive for CD3, CD8, and L-26 ( Fig. 4 ). Polymerase chain reaction (PCR) amplification did not show human herpes simplex viruses in the skin biopsy specimen, and culture was also negative. Figure 1 Open in figure viewer PowerPoint (a) Clinical aspect: erythematous papulonodules on the face. (b) Clinical aspect: erythematous papulonodules of the upper part of the trunk 相似文献
A 57-year-old man had had deformities of all ten fingernails for one and a half years before presentation and deformities of all ten toenails for the previous six months. The surfaces of the nails were rough, with excessive longitudinal striations. The bases of the nails were slightly hypertrophic, and the tips were atrophic and itchy. A longitudinal nail biopsy including the nail matrix revealed the typical histology of lichen planus. Reticulated pigmentation, maceration, and erosion on the buccal mucous membrane were also discovered. Histological analysis of the buccal mucous membrane revealed lichen planus intermingled with eosinophils. Immunological blood analysis revealed elevated CD4+ T cells and CD4/CD8 ratio. He worked as a tinsmith and had dental metal. The metal series patch test revealed positive reactions to chromate and tin. Treatment with systemic steroids was quite effective in treating the nail lesions. 相似文献
Examination of biopsy samples from 62 patients with--or with suspected--cutaneous T-cell lymphoma (CTCL) revealed 2 cases in which the neoplastic cells were positive for the T-cell receptor (TCR) gamma delta complex. One patient had mycosis fungoides and 1 patient had a pleomorphic lymphoma of medium and large-cell type. Both cases showed aggressive courses with dissemination to internal organs and short survival times. The phenotypic examination showed that the neoplastic cells were positive with TCR delta 1, CD3, CD25, CD29, CD45R0 and CD54. No staining was seen with antibodies against framework determinants or variable regions on the TCR alpha beta heterodimer. Negative reactions were also seen with CD4, CD8, CD5, CD7, CD16, CD30 and CD57. It is concluded that rare CTCL express TCR gamma delta chains. These malignancies may originate from the TCR gamma delta-positive T cells seen in normal skin, and it is possible that their recognition may be important for clinical reasons. 相似文献
