Acta Dermato-Venereologica 98-10CompleteContent | Page 10

951 CLINICAL REPORT Treatment of Early Folliculotropic Mycosis Fungoides with Special Focus on Psoralen plus Ultraviolet A Iris AMITAY-LAISH 1,2 , Hadas PRAG-NAVEH 1 , Adam DALAL 1 , Lev PAVLOVSKY 1,2 , Meora FEINMESSER 2,3 and Emmilia HODAK 1,2 Department of 1 Dermatology and 3 Pathology, Rabin Medical Center - Beilinson Hospital, Petach Tikva, and 2 Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel Data on the treatment of early folliculotropic myco- sis fungoides, a recently defined clinicopathological subgroup of folliculotropic mycosis fungoides with an indolent course, is limited. Treatment outcomes were studied in a retrospective cohort of 47 adults with early folliculotropic mycosis fungoides, with a focus on psoralen plus ultraviolet A (PUVA) monotherapy, including dosimetric data, and the findings were com- pared with data for PUVA in 18 adults with early-clas- sic mycosis fungoides. PUVA was given to 27 patients with early folliculotropic mycosis fungoides: 70% achieved complete response and 26% partial response. Significantly more treatments were needed to achieve complete response in stage IB compared with stage IA. There was no significant difference in the com- plete response rate from classic plaque-stage disease, although the early folliculotropic mycosis fungoides group required more treatments to achieve complete response, and a higher cumulative dose of UVA. Thus, PUVA is an effective treatment for early folliculotropic mycosis fungoides. Its complete response rate might be equal to early-classic mycosis fungoides; however, a longer induction phase is needed to achieve comple- te response. Key words: folliculotropic; mycosis fungoides; psoralen and ul- traviolet A; PUVA; phototherapy; cutaneous T-cell lymphoma. Accepted Aug 7, 2018; Epub ahed of print Aug 7, 2018 Acta Derm Venereol 2018; 98: 951–955. Corr: Iris Amitay-Laish, Department of Dermatology, Rabin Medical Cen- ter – Beilinson Hospital, Petach Tikva 4941492, Israel. E–mail: amitay- [email protected] F olliculotropic mycosis fungoides (FMF) has long been considered an aggressive variant of mycosis fungoides (MF) (1, 2). However, our experience has shown that FMF presents with 2 distinct patterns of clinicopathological features representing an early variant and an advanced tumour variant. Their prognostic impli- cations differ, with early FMF following a more indolent course (3). These findings were validated by the Dutch Cutaneous Lymphoma Group (4). Traditionally, treatment recommendations for FMF have followed those for classic tumour-stage MF. Re- cently, the Dutch Cutaneous Lymphoma Group reported a response of patients with early FMF to skin-targeted therapy, and specifically psoralen plus ultraviolet A (PUVA) (5). In fact, in a few earlier publications (6–10) SIGNIFICANCE Since data on the treatment of early folliculotropic mycosis fungoides is limited, we studied the treatment outcomes in patients with this subset of mycosis fungoides. The ma- jority of patients responded to nonaggressive treat­ments and had an indolent course. Systemic PUVA was the most common first line treatment, associated with a high re- sponse rate, whereas narrowband UVB was found to have low efficacy. Although PUVA, according to our study, might be an equally effective treatment as for early classic my- cosis fungoides, significantly more exposures were needed to achieve complete response in early folliculotropic mycosis fungoides. PUVA was found to be beneficial in a subset of cases of FMF, which, based on recent insights into the disease (3, 4), can be redefined as early FMF. In addition, in a small study of adults and children with clinicopathological features that were defined by us as those of early FMF, we observed a beneficial effect of skin-targeted therapy with PUVA bath and, in few cases, systemic PUVA (11, 12). Yet, data on the treatment outcome of PUVA in early FMF is still scarce, and there is no data on the specific protocol including the dosimetric parameters needed to achieve optimal response. Early-classic MF is histologically characterized by the infiltration of lymphocytes into the epidermis, the dermo–epidermal junction, and the papillary dermis (13). By contrast, early FMF is characterized by epi­ thelial infiltration to the follicle (3). The different and frequently deeper location of the dominant pathological focus in early FMF (3) may make it less accessible to skin-targeted therapy than early-classic MF. No compari- sons of the efficacy of PUVA have been reported between early FMF and early-classic MF. The aim of the present study was to assess the response of early FMF to initial treatments, especially systemic PUVA monotherapy, with an emphasis on dosimetric data, and to compare the findings with those for early- classic MF. PATIENTS AND METHODS A retrospective cohort design was used. The study group con- sisted of consecutive patients treated for early FMF from January 1995 to June 2016 at the tertiary Cutaneous Lymphoma Clinic of Rabin Medical Center. FMF was defined according to the World This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica. doi: 10.2340/00015555-3013 Acta Derm Venereol 2018; 98: 951–955