R Fernández-de-Misa1, B Hernández-Machín2, O Servitje3, F Valentí-Medina3, L Maroñas-Jiménez4, P L Ortiz-Romero4, J Sánchez Schmidt5, R M Pujol5, F Gallardo5, I Pau-Charles6, M P García Muret7, S Pérez Gala8, C Román9, J Cañueto9, L Blanch Rius10, R Izu10, A Ortiz-Brugués11, R M Martí11, M Blanes12, M Morillo13, P Sánchez14, Y Peñate15, J Bastida16, A Pérez Gil17, I Lopez-Lerma18, C Muniesa3, T Estrach6. 1. Department of Dermatology and Research Unit, Hospital Universitario Nuestra Señora de Candelaria, Santa Cruz de Tenerife, Spain. 2. Department of Dermatology, Clínica Buenaderma, Las Palmas de Gran Canaria, Spain. 3. Department of Dermatology, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain. 4. Department of Dermatology, Hospital Universitario 12 de Octubre, i+12 Research Institute, Universidad Complutense Madrid, Madrid, Spain. 5. Department of Dermatology, Hospital del Mar, Universitat Autónoma de Barcelona, Barcelona, Spain. 6. Department of Dermatology, Hospital Clínico, University of Barcelona, IDIBAPS, Barcelona, Spain. 7. Department of Dermatology, Hospital Santa Creu i Sant Pau, UAB, Barcelona, Spain. 8. Department of Dermatology, Hospital Universitario Ramón y Cajal, Madrid, Spain. 9. Department of Dermatology, Hospital Universitario de Salamanca, Salamanca, Spain. 10. Department of Dermatology, Hospital de Basurto, Bilbao, Spain. 11. Department of Dermatology, IRBLleida, Hospital Universitari Arnau de Vilanova, Lleida, Spain. 12. Department of Dermatology, Hospital General Universitario de Alicante, Alicante, Spain. 13. Department of Dermatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain. 14. Department of Dermatology, Hospital de León, León, Spain. 15. Department of Dermatology, Complejo Hospitalario Universitario Insular Materno-Infantil, Gran Canaria, Las Palmas de Gran Canaria, Spain. 16. Department of Dermatology, Hospital Universitario Dr. Negrín, Las Palmas de Gran Canaria, Spain. 17. Department of Dermatology, Hospital Virgen de Valme, Sevilla, Spain. 18. Department of Dermatology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
Abstract
BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical 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 10 months (95% CI 6-13 months), 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 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; 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. CONCLUSIONS: Current 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.
BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical 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 10 months (95% CI 6-13 months), 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 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; 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. CONCLUSIONS: Current 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.