Pedram Gerami1, Steve Rosen, Timothy Kuzel, Susan L Boone, Joan Guitart. 1. Department of Dermatology, Northwestern University Feinberg School of Medicine, and The Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, Illinois, USA.
Abstract
OBJECTIVES: To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group. SETTING: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group. PATIENTS: Forty-three patients with FMF were included in the study and compared with 43 age- and stage-matched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time. RESULTS: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (< or =IIA) had a 10-year survival of 82%, which took a steep drop off to 41% by 15 years. Patients with late-stage disease (> or =IIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage. CONCLUSIONS: The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skin-directed therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.
OBJECTIVES: To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group. SETTING: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group. PATIENTS: Forty-three patients with FMF were included in the study and compared with 43 age- and stage-matched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time. RESULTS: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (< or =IIA) had a 10-year survival of 82%, which took a steep drop off to 41% by 15 years. Patients with late-stage disease (> or =IIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage. CONCLUSIONS: The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skin-directed therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.
Authors: Julia J Scarisbrick; H Miles Prince; Maarten H Vermeer; Pietro Quaglino; Steven Horwitz; Pierluigi Porcu; Rudolf Stadler; Gary S Wood; Marie Beylot-Barry; Anne Pham-Ledard; Francine Foss; Michael Girardi; Martine Bagot; Laurence Michel; Maxime Battistella; Joan Guitart; Timothy M Kuzel; Maria Estela Martinez-Escala; Teresa Estrach; Evangelia Papadavid; Christina Antoniou; Dimitis Rigopoulos; Vassilki Nikolaou; Makoto Sugaya; Tomomitsu Miyagaki; Robert Gniadecki; José Antonio Sanches; Jade Cury-Martins; Denis Miyashiro; Octavio Servitje; Cristina Muniesa; Emilio Berti; Francesco Onida; Laura Corti; Emilia Hodak; Iris Amitay-Laish; Pablo L Ortiz-Romero; Jose L Rodríguez-Peralto; Robert Knobler; Stefanie Porkert; Wolfgang Bauer; Nicola Pimpinelli; Vieri Grandi; Richard Cowan; Alain Rook; Ellen Kim; Alessandro Pileri; Annalisa Patrizi; Ramon M Pujol; Henry Wong; Kelly Tyler; Rene Stranzenbach; Christiane Querfeld; Paolo Fava; Milena Maule; Rein Willemze; Felicity Evison; Stephen Morris; Robert Twigger; Rakhshandra Talpur; Jinah Kim; Grant Ognibene; Shufeng Li; Mahkam Tavallaee; Richard T Hoppe; Madeleine Duvic; Sean J Whittaker; Youn H Kim Journal: J Clin Oncol Date: 2015-10-05 Impact factor: 44.544
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