Details

Autor(en) / Beteiligte

• Santen, S.
• Doorn, R.
• Neelis, K.J.
• Daniëls, L.A.
• Horváth, B.
• Bruijn, M.S.
• Sanders, C.J.G.
• Rossum, M.M.
• Haas, E.R.M.
• Veraart, J.C.J.M.
• Bekkenk, M.W.
• Vermeer, M.H.
• Willemze, R.

Titel

Recommendations for treatment in folliculotropic mycosis fungoides: report of the Dutch Cutaneous Lymphoma Group

Ist Teil von

• British journal of dermatology (1951), 2017-07, Vol.177 (1), p.223-228

Ort / Verlag

England: Oxford University Press

Erscheinungsjahr

2017

Quelle

Wiley Online Library Journals Frontfile Complete

Beschreibungen/Notizen

• Summary Background Folliculotropic mycosis fungoides (FMF) is an aggressive variant of mycosis fungoides (MF) and generally less responsive to standard skin‐directed therapies (SDTs). Recent studies distinguished indolent (early‐stage FMF) and more aggressive (advanced‐stage FMF) subgroups. The optimal treatment for both subgroups remains to be defined. Objectives To evaluate initial treatment results in patients with early‐ and advanced‐stage FMF. Methods A study was undertaken of 203 patients (84 early‐stage, 102 advanced‐stage, 17 extracutaneous FMF) included in the Dutch Cutaneous Lymphoma Registry between 1985 and 2014. Type and results of initial treatment were retrieved from the Dutch Registry. Main outcomes were complete remission (CR); sustained complete remission; partial remission (PR), > 50% improvement; and overall response (OR; CR + PR). Results Patients with early‐stage FMF were treated with nonaggressive SDTs in 67 of 84 cases resulting, respectively, in CR and OR of 28% and 83% for monotherapy topical steroids, 0% and 83% for ultraviolet B (UVB), and 30% and 88% for psoralen plus ultraviolet A (PUVA). In patients with advanced‐stage FMF these SDTs were less effective (combined CR and OR 10% and 52%, respectively). In patients with advanced‐stage FMF local radiotherapy (CR 63%; OR 100%), total skin electron beam irradiation (CR 59%; OR 100%) and PUVA combined with local radiotherapy (CR 5%, OR 75%) were most effective. Conclusions The results of the present study demonstrate that not all patients with FMF should be treated aggressively. Patients with early‐stage FMF may benefit very well from standard SDTs also used in early‐stage classic MF and have an excellent prognosis. What's already known about this topic? Folliculotropic mycosis fungoides (FMF) has a worse prognosis and is less responsive to skin‐directed therapies (SDTs) compared with classic mycosis fungoides (MF). Recent studies distinguished indolent (early‐stage FMF) and more aggressive (advanced‐stage FMF) subgroups. The optimal treatment for both subgroups needs still to be defined. What does this study add? Treatment recommendations for different subgroups of FMF are formulated. Treatment response of patients with early‐stage FMF is superior to that of patients with advanced‐stage FMF. Patients with early‐stage FMF may benefit very well from standard SDTs also used in classic MF and have an excellent prognosis. Respond to this article Linked Comment: Bagot. Br J Dermatol 2017; 177:17–18