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INTRODUCTION/BACKGROUND: Advanced-stage mycosis fungoides (MF) and Sézary syndrome (SS) have poor prognosis with median survivals of less than 5 years. Although a variety of treatments are approved for MF/SS patients, durable complete remissions (CR) are rare. PATIENTS AND METHODS: Advanced-stage MF or SS patients who achieved CR and maintained in CR or stage IA for more than 10 years were identified by a retrospective search of the principal investigator's database. RESULTS: Of 2266 patients diagnosed with MF or SS, 23 patients with advanced-stage MF/SS (6 IIB, 1 IIIB, 5 IVA1, 3 IVA2, 8 IVB) who achieved CR and maintained in CR or stage IA for ≥ 10 years were identified. As final/curative treatment, 11 patients underwent allogeneic stem cell transplantation (SCT). Most patients presented at young age, underwent SCT with reduced intensity conditioning regimen, had matched related donors, and controllable post-transplant graft versus host disease. Eleven patients were treated with TSEB as part of combined modality protocol in 2 patients and debulking therapy before allogeneic SCT in 9 patients. Five stage IIB patients achieved CR with radiotherapy. Four patients with blood involvement were treated with extracorporeal photopheresis (ECP) in combination with long-term antibiotics and immunomodulatory agents. Long-term antibiotics were given to 14 patients. CONCLUSION: TSEB followed by allogeneic SCT, radiotherapy, ECP plus long-term antibiotics and immunomodulatory agents were the most common curative/final treatments found in our patients. We are reporting the details of our long-term complete responders' treatment course in the hopes of obtaining more cure responses in the future.

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BACKGROUND: Mycosis fungoides (MF) in children is a rare disease and there are limited data regarding the behavior of the disease in this age group. We aimed to collect additional data to better understand the clinicopathologic features of MF in children. MATERIALS AND METHODS: This study was a retrospective analysis of pediatric MF patients (diagnosed at age 0 to 18 y). RESULTS: Thirteen pediatric patients with MF were identified. Female predominance was observed with a ratio of 1.6:1. Median values for age of onset of skin lesions and age at the time of histologic diagnosis were 5 and 12 years, respectively. All patients had early stage (stage IA to IIA) of MF at the time of diagnosis. Hypopigmented MF comprised 77% of all study patients, followed by classic MF (15%) and pagetoid reticulosis (8%). The lower extremity (especially proximal leg) followed by trunk and upper extremity were most commonly affected sites. Seven of 9 patients who had available immunohistochemistry data showed CD8 + predominance. Five of 8 patients whose follow-up data was available, achieved complete response with narrowband ultraviolet B treatment, while 2 and 1 had near complete response and partial response, respectively. CONCLUSIONS: Our study demonstrated female sex and CD8 + profile predominance. Hypopigmented MF constituted the majority of cases. We observed good responses with narrowband ultraviolet B treatment.

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Because Malassezia folliculitis (MF) may clinically mimic acne vulgaris (AV), patients usually receive unnecessary and prolonged antibiotic treatment. We aimed to determine the prevalence of MF among patients with AV, and to evaluate its response to antifungal treatment. A total of 217 patients with AV underwent cytologic examination for the presence of Malassezia yeasts. Samples were obtained from lesional and nonlesional skin and stained with May-Grünwald-Giemsa. MF was diagnosed if there were more than six spores in one microscopic field (at ×400 magnification). A modified "lesion-counting" method was used to assess the clinical severity of acne. Treatment included oral itraconazole (2×100 mg daily) and topical ketaconazole for 4 weeks. Fifty-five (25.3%) patients were diagnosed with MF; of these, 38 (69.1%) completed the antifungal treatment. The lesions decreased by 50% or more in 26 (68.4%) of the patients who completed the antifungal treatment, which reduced the number of closed comedones/comedolike or molluscoid papules and inflammatory papules. The average number of spores in lesional samples was significantly decreased after treatment (P=<.0005). We observed that MF can present with AV-like lesions, or the two diseases may coexist. Cytology is helpful for making the correct diagnosis and providing proper management of MF.

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Several chemotherapy agents have shown efficacy in the treatment of mycosis fungoides (MF). In the literature, there is limited data on the use of single agent etoposide for MF. We aimed to retrospectively review our experience with single agent etoposide in the treatment of advanced-stage or refractory early-stage MF with focus on analyzing its efficacy and safety. We included 13 MF patients who were treated with single agent etoposide. Patients were identified through the Cutaneous T Cell Lymphoma Database of Indiana University that involves patients treated from 2006 to 2016. Overall nine patients (69%) responded to treatment. No complete response was identified. Median time to response was 12.5 weeks (range: 6-25.4). Median duration of response was 43 weeks (range: 5-60) and median time to treatment failure was 31.3 weeks (range: 12.4-230). Hematological toxicity was observed in eight patients including two patients with grade 4 neutropenia and/or lymphopenia leading to sepsis. Higher doses of etoposide were significantly correlated with higher grades of anemia, neutropenia or lymphopenia (p < .05). Our study demonstrates that etoposide is an effective treatment for MF and may be considered in selected patients with progressive MF who have failed other treatments.

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