Abstract

Introduction: Phototherapy, particularly psoralen plus ultraviolet A (PUVA) and narrowband ultraviolet B (nbUVB), is a mainstay in the treatment of Mycosis Fungoides (MF).
 Methods: We analyzed outcomes of MF patients treated for the first time with either PUVA or nbUVB at the Photodermatology Unit, in the Department of Dermatology of Centro Hospitalar Universitário do Porto, from 2007 to 2017. The primary outcome was response to treatment. The secondary outcome was disease relapse free survival.
 Results: From a total number of 34 patients with MF identified, nine (26.5%) patients were diagnosed at stage IA and 25 (73.5%) patients were diagnosed at stage IB. Of these patients, 30 (88.2%) were treated with PUVA and four (11.8%) with nbUVB. Overall, 24 (80%) patients treated with PUVA and two (50%) patients treated with nbUVB had complete responses (CR). Relapse was observed in 17 (70.8%) PUVA treated patients and two (100%) nbUVB treated patients. Median disease relapse free survival was 12 months for PUVA and 6 months for nbUVB.
 Conclusion: PUVA is an effective therapy in early MF. Narrowband UVB could be a valid therapeutic alternative for these patients. However, the small size of nbUVB group in our study limits the conclusions about its efficacy.

Highlights

  • Primary cutaneous lymphomas are defined as non-Hodgkin lymphomas present in the skin without evidence of extracutaneous disease at the time of diagnosis.[1]

  • The first publication on the use of plus ultraviolet A light (PUVA) phototherapy for the treatment of Mycosis fungoides (MF) was in 1976,8 and since more than 30 studies of treatment with PUVA in patients with MF have been published in English

  • 80% of patients treated with PUVA achieved complete responses (CR) and 3.3% of patients achieved partial response (PR)

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Summary

INTRODUCTION

Primary cutaneous lymphomas are defined as non-Hodgkin lymphomas present in the skin without evidence of extracutaneous disease at the time of diagnosis.[1] In the western world, cutaneous T cell lymphomas (CTCL) account to about 75% to 80% of all primary cutaneous lymphomas. Mycosis fungoides (MF) is the most common type of CTCL, with 75% of affected patients being at early stage during initial diagnosis.[2,3] Skin-directed therapies, including phototherapy, are a mainstay treatment for most patients with early stage MF.[4] Both narrow-band ultraviolet B light (nbUVB) phototherapy and psoralen plus ultraviolet A light (PUVA) photochemotherapy, have recognized efficacy in MF and are widely used treatments for early stages.[5] Despite the large use of phototherapy on early stage MF, exact mechanisms of action are currently unknown. The theoretical advantages of PUVA over nbUVB are in terms of reduced erythemogenic activity and better penetration towards deeper dermal layers, up to hypodermis and deepest adnexal structures, where can affect lymphocytes as well as other resident skin cells, such as fibroblasts, dendritic cells, mast cells, endothelial cells or macrophages.[5,6] We conducted a retrospective study to evaluate the efficacy of phototherapy, PUVA and nbUVB, in early stage MF, in a tertiary hospital of Porto, Portugal

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