First-line treatment in lymphomatoid papulosis: a retrospective multicentre study.

R Fernández-de-Misa,B Hernández-Machín, O Servitje, F Valentí-Medina, L Maroñas-Jiménez,P L Ortiz-Romero, J Sánchez Schmidt,R M Pujol, F Gallardo, I Pau-Charles,M P García Muret, S Pérez Gala,C Román,J Cañueto, L Blanch Rius, R Izu, A Ortiz-Brugués,R M Martí, M Blanes,M Morillo,P Sánchez,Y Peñate, J Bastida,A Pérez Gil,I Lopez-Lerma, C Muniesa, T Estrach

CLINICAL AND EXPERIMENTAL DERMATOLOGY(2018)

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摘要
BackgroundData regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AimTo assess the daily clinical practice approach to LyP and the response to first-line treatments. MethodsThis was a retrospective study enrolling 252 patients with LyP. ResultsTopical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR=1.76; 95% CI 1.16-2.11). Overall median time to CR was 10months (95% CI 6-13months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P>0.05). Overall estimated median disease-free survival (DFS) was 11months (95% CI 9-13months) but DFS for patients treated with phototherapy was 23months (95% CI 10-36months; P<0.03). Having the Type A LyP variant (RR=2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR=5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. ConclusionsCurrent epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.
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