Paediatric mycosis fungoides – characteristics, management and outcomes with particular focus on the folliculotropic variant

Abstract

Background The literature on paediatric mycosis fungoides (MF) and especially its folliculotropic variant (FMF) is sparse. Objectives To describe the clinical manifestations, treatments, outcomes and long‐term course of paediatric MF, including FMF. Methods A retrospective analysis was conducted of all consecutive MF patients diagnosed at ≤18 years attending two medical centres in 1995–2015. Results The cohort included 71 patients, all but two of whom had early‐stage disease: hypopigmented (55%), folliculotropic (42%) and classical MF (39%), alone or in combination. The head and neck area were involved in 43% of patients with early‐stage FMF compared to 12% of the non‐FMF group (P = 0.004). There was no difference in the involvement of other body areas between the groups. Pruritus, although mild, was more often recorded among patients with early‐stage FMF compared to non‐FMF (58% vs. 29%, respectively, P = 0.02). Complete response (CR) was achieved in 60 of the 69 patients with early‐stage MF (87%) after an average of 1.8 treatment modalities. NBUVB was the most administered treatment to non‐FMF patients with CR rates of 63% vs. 29% of FMF patients (P = 0.04). Systemic/bath PUVA and UVA+NBUVB were the most administered treatments to FMF patients with CR rates of 60% vs. 81% for non‐FMF patients (P = 0.17). During a mean follow‐up of 9.2 years (range 1–24), stage progression was observed in four (6%) of the patients with early‐stage disease, two of whom (all FMF) to advanced stage. Conclusions Paediatric MF presents as an early‐stage disease with over‐representation of hypopigmented and FMF variants. NBUVB and UVA‐based therapies yield good response rates in non‐FMF and FMF patients, respectively. Disease course is indolent, and even on relatively long follow‐up, it has a very low progression rate from early to advanced‐stage disease, occurring in patients with FMF. We propose a treatment algorithm for paediatric MF.

Hypopigmented Mycosis Fungoides: A Clinicopathological Review of 32 Patients

“…In terms of immunohistochemical results, hMF has similar histological presentation with classic MF, but hMF is identified by CD8 positive profile instead of CD4. 3 Currently, there have been more hMF cases reported worldwide. However, there is less literature to describe the general condition of hMF in the Asian population, especially Chinese patients.…”

Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides

“…hMF is an unusual subtype of MF that is typically more prevalent in younger individuals with darker skin types and has a better prognosis than other types of MF ( 2 , 11 , 12 ). hMF is histopathologically similar to classical MF, but IHC reveals a predominance of CD8+ T cells.…”

Folliculotropic Mycosis Fungoides: Current Guidance and Experience from Clinical Practice

“…As for special populations, the FMF subtype has been recently found to be over-represented in solid organ transplant recipients and in paediatric patients, compared with other CTCL variants. 6 , 7 Recent studies reported 10-year survival rates of 72% in skin-limited early stages, 28% in skin-limited advanced stages, and 2% in FMF with extracutaneous localizations at first presentation. 5 As for therapeutic options, the latest guidelines on MF agree on a stage-driven strategy, in consideration of clinical presentation, symptom burden and patient’s comorbidities.…”