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      A Rare Presentation of Mycosis Fungoides Mimicking Psoriasis Vulgaris

      research-article
      , , *
      Case Reports in Dermatology
      S. Karger AG
      Mycosis fungoides, Cutaneous lymphoma, Psoriasis

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          Abstract

          Mycosis fungoides (MF) is an uncommon primary cutaneous lymphoma with a wide spectrum of clinicopathological manifestations. Diagnosis can be difficult in its early stages given the considerable overlap with more common benign dermatoses. We report an unusual case of MF in a 52-year-old male presenting with psoriasiform plaques on the palms and the soles who rapidly developed additional lesions on the scalp, limps and trunk. Punch biopsy of the face was obtained for routine histology and immunohistochemical stains. Chest X-ray, total body computed tomography scanning and excisional biopsy of the inguinal lymph node were performed. Review of the face biopsy revealed a diffuse dermal infiltrate containing a high number of atypical lymphocytes showing a CD3+, CD4+, CD45RO+, CD8–, CD20– immunophenotype and epidermotropism. Findings were consistent with tumor stage MF (stage IIB, T3 N1 M0). We report a rare presentation of MF mimicking psoriasis vulgaris.

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          Most cited references15

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          EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome.

          Several reviews and guidelines on the management of mycosis fungoides and Sézary syndrome (MF/SS) have been published; however, treatment strategies for patients with MF/SS vary from institution to institution and no European consensus has yet been established. There are few phase III trials to support treatment decisions for MF/SS and treatment is often determined by institutional experience. In order to summarise the available evidence and review 'best practices' from each national group, the European Organisation for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Task Force met in September 2004 to establish European guidelines for the treatment of MF/SS. This article reviews the treatment regimens selected for inclusion in the guidelines and summarises the clinical data for treatments appropriate for each stage of MF/SS. Guideline recommendations are presented according to the quality of supporting data, as defined by the Oxford Centre for Evidence-Based Medicine. Skin-directed therapies are the most appropriate option for early-stage MF/SS and most patients can look forward to a normal life expectancy. Patients with advanced disease should be encouraged to participate in clinical trials and maintenance of quality of life should be paramount.
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            Mycosis fungoides and Sézary syndrome.

            Mycosis fungoides and Sézary syndrome are the most common of the cutaneous T-cell lymphomas, which are a heterogeneous group of neoplasms that affect the skin as a primary site. Although the aetiologies of mycosis fungoides and Sézary syndrome are unknown, important insights have been gained in the immunological and genetic perturbations that are associated with these diseases. Unlike some B-cell lymphomas, cutaneous T-cell lymphomas as a group are rarely if ever curable and hence need chronic-disease management. New approaches to treatments are being investigated and include biological and cytotoxic drugs, phototherapy, and monoclonal antibodies that are directed towards novel molecular targets. New molecular technologies such as complementary-DNA microarray have the potential to increase the accuracy of diagnosis and provide important prognostic information. Treatments can be combined to greatly improve clinical outcome without substantially increasing toxic effects in advanced disease that is otherwise difficult to treat. Although present treatment strategies are generally not curative, there is hope that experimental treatments, particularly immunotherapy, might eventually reverse or suppress the abnormalities of mycosis fungoides and Sézary syndrome to the point at which they become non-life-threatening, chronic diseases.
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              Clinicopathological spectrum of mycosis fungoides.

              Cutaneous lymphomas represent a heterogeneous group of T-, NK- and B-cell neoplasms, with mycosis fungoides (MF) being the most common subtype. MF has a plethora of clinicopathological manifestations. Many variants of this lymphoma differ substantially from the 'classical' Alibert-Bazin disease and are therefore sometimes referred to as 'atypical' forms of the disease. This review addresses the whole clinicopathological spectrum of mycosis fungoides with respect to epidemiology, clinical, histopathological, immunophenotypic and genotypic features and the clinical course and prognosis of its variants: classical, erythrodermic, follicular, syringotropic, bullous/vesicular, granulomatous, poikilodermic, hypo- and hyperpigmented, unilesional, palmoplantar, hyperkeratotic/verrucous, vegetating/papillomatous, ichthyosiform, pigmented purpura-like, pustular and mucosal involvement in MF.
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                Author and article information

                Journal
                Case Rep Dermatol
                CDE
                Case Reports in Dermatology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )
                1662-6567
                Jan-Dec 2009
                27 October 2009
                27 October 2009
                : 1
                : 1
                : 60-65
                Affiliations
                Department of Dermatology, University of Palermo, Palermo, Italy
                Author notes
                *Maria Rita Bongiorno, MD, Department of Dermatology, University of Palermo, Via del Vespro 131, IT–90127 Palermo (Italy), Tel. +39 091 655 4001, Fax +39 091 655 4002, E-Mail istderm@ 123456unipa.it
                Article
                cde0001-0060
                10.1159/000249148
                2895213
                20652117
                b4e257db-9d40-4f91-9a11-ab2ffa8b1672
                Copyright © 2009 by S. Karger AG, Basel

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License ( http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com as well as the terms of this license are included in any shared versions.

                History
                Page count
                Figures: 2, Tables: 1, References: 15, Pages: 6
                Categories
                Case Reports in Dematology

                Dermatology
                mycosis fungoides,cutaneous lymphoma,psoriasis
                Dermatology
                mycosis fungoides, cutaneous lymphoma, psoriasis

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