1/16. 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 = 1keywords = erythroderma (Clic here for more details about this article) |
2/16. 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 = 0.5keywords = erythroderma (Clic here for more details about this article) |
3/16. 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 = 0.5keywords = erythroderma (Clic here for more details about this article) |
4/16. Erythrodermic syringotropic cutaneous T-cell lymphoma.Syringotropic cutaneous T-cell lymphoma (CTCL) is a rare localized variant of CTCL, characterized histologically by eccrine gland and ductal hyperplasia surrounded by a dense syringotropic lymphocytic infiltrate. Previously reported only in men, we describe the first woman with syringotropic CTCL. Unusually, she presented with erythroderma, cutaneous nodules, poikilodermatous patches, widespread alopecia and lymphadenopathy.- - - - - - - - - - ranking = 0.5keywords = erythroderma (Clic here for more details about this article) |
5/16. Monitoring the decrease of circulating malignant T cells in cutaneous T-cell lymphoma during photopheresis and interferon therapy.BACKGROUND: The prognosis of patients with stage IV cutaneous T-cell lymphoma (CTCL) is grim and therapeutic options are limited. Treatment of advanced-stage CTCL is aimed at suppressing the dominant T-cell clone, which is typically present in the skin, peripheral blood, and lymph nodes. OBSERVATIONS: We detected the expansion of 1 T-cell clone expressing the T-cell receptor V beta 14 in the peripheral blood of a patient with stage IVA CTCL. Before initiation of combination therapy with photopheresis and low-dose interferon alpha, the dominant T-cell clone represented 84% of the total T-cell population. After successful therapy, this clone showed a dramatic decrease to 6% of the T-cell population after 6 months of treatment. This reduction in the percentage of the malignant T-cell population in response to therapy was paralleled by clinical skin improvement from initial generalized erythroderma to undetectable skin disease. CONCLUSIONS: This case demonstrates that response to combination treatment with photopheresis and low-dose interferon alpha in patients with advanced CTCL may be accurately and quantitatively followed up by monitoring the percentage of the malignant T-cell clone (when identifiable) within the total circulating T-cell population by flow cytometry.- - - - - - - - - - ranking = 0.5keywords = erythroderma (Clic here for more details about this article) |
6/16. Ofuji papuloerythroderma evolving to cutaneous T-cell lymphoma.Ofuji papuloerythroderma is an uncommon entity of unknown aetiology, characterized by a pruritic eruption of widespread, red-brown, flat papules that leads to spare skin folds. A number of cases have been described associated with tumour pathology, mainly cutaneous T-cell lymphomas. We report a new case of Ofuji papuloerythroderma evolving to cutaneous T-cell lymphoma in an 85-year-old woman who had been previously diagnosed with papuloerythroderma 7 years previously.- - - - - - - - - - ranking = 3.5keywords = erythroderma (Clic here for more details about this article) |
7/16. role of slit skin smear examination in cutaneous T-cell lymphomas and other chronic dermatoses.The purpose of this study was to evaluate the utility of slit-skin smear examination in the diagnosis of various chronic dermatoses. The study included 24 patients with chronic dermatoses who presented to the skin outpatient department. Ten patients had idiopathic erythroderma, seven were diagnosed with airborne contact dermatitis, four had cutaneous T-cell lymphoma, two had chronic actinic dermatoses, and a single patient had panniculitis. Slit skin smears were obtained from all patients, stained with Leishman stain, and examined under microscope. Out of 24 patients, all four cases of cutaneous T-cell lymphoma showed abnormal cells suggestive of lymphomatous infiltration, two patients with airborne contact dermatitis showed reactive lymphocytes, and two idiopathic erythroderma cases showed numerous eosinophils in the smear. Slit skin smear examination is a simple rapid, decisive test in the diagnosis of cutaneous T-cell lymphoma. It is a useful screening test, especially in sezary syndrome and diseases with specific skin infiltrate.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
8/16. Clonal chronic lymphocytic leukaemia-like B lymphocytes in the blood of patients with cutaneous T-cell disorders.A population of B cells with characteristics of chronic lymphocytic leukaemia was found in the peripheral blood of four patients who presented with cutaneous infiltration of atypical CD4 T cells with cerebriform nuclei. The B cells had a low density of immunoglobulin on their surface membrane, expressed CD5-positivity, and showed monoclonality based on the restriction to either kappa or lambda light chains. In one patient with tumourous pleiomorphic CD4 CD30- T-cell lymphoma of the skin, it was the first manifestation of a concomitant B-cell non-Hodgkin's lymphoma of low-grade malignancy. In three other patients with reactive atypical T-cell erythroderma, there was no evidence for the coexistence of a B-cell malignancy. The number of CD5 B cells decreased in two erythroderma patients with clinical remission of the cutaneous lesions. It is speculated that the presence of a monoclonal B cell population in patients with T-cell disorders of the skin is due either to a reactive process possibly conferring some protective effect, or a response to an unknown stimulus produced by the T cells.- - - - - - - - - - ranking = 1keywords = erythroderma (Clic here for more details about this article) |
9/16. De novo development of psoriatic plaques in patients receiving interferon alfa for treatment of erythrodermic cutaneous T-cell lymphoma.Presumably because of its potent immunomodulatory activity, the use of interferon has led to the development of autoimmune disease in susceptible individuals. Because psoriasis is considered to be, in part, an autoimmune phenomenon, it is plausible that interferon may influence disease activity. We describe the development of psoriatic plaques in two patients without a history of this disease while they were receiving interferon alfa and extracorporeal photochemotherapy for erythrodermic cutaneous T-cell lymphoma. Paradoxically, in both patients the erythroderma resolved with subsequent de novo onset of psoriasis. This clinical sequence provides support for disparate immune mechanisms in the pathogenesis of these disorders, both of which are typified by lymphoid infiltrates. A review of the literature reveals that all forms of interferons have been associated with the exacerbation of psoriatic plaques, but that only interferon alfa has induced de novo development of psoriasis.- - - - - - - - - - ranking = 0.5keywords = erythroderma (Clic here for more details about this article) |
10/16. An aggressive spindle cell squamous carcinoma arising in a patient with long-standing erythroderma.Spindle cell squamous carcinoma (SCSC) of the left hand arising in a patient with long-standing erythroderma is reported. Histopathologically, spindle shaped atypical cells were observed neighboring the cells of well differentiated squamous cell carcinoma. These two types of tumor cells, spindle cells and well differentiated cells, were present side by side and merged into each other. The erythroderma had been present for over 20 years, and both clinical and histopathological findings suggested cutaneous T cell lymphoma, but were not diagnostic for mycosis fungoides, sezary syndrome, or adult T cell lymphoma. flow cytometry of peripheral blood cells showed a low CD4/CD8 ratio which suggested impaired T cell function. Multiple metastases of SCSC occurred in a short period and the patient died ten months after his first visit to us. The aggressive course of this case was unusual, and may be due to immunological abnormalities associated with the long standing erythroderma with impaired T cell function.- - - - - - - - - - ranking = 3.5keywords = erythroderma (Clic here for more details about this article) |
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