Folliculotropic mycosis fungoides: Clinical characteristics, treatments, and long‐term outcomes of 53 patients in a tertiary hospital

Abstract

Folliculotropic mycosis fungoides (FMF) is characterized by a broad clinical spectrum and worse prognosis compared to classical MF. This study aimed to evaluate the clinical characteristics, treatment modalities and long‐term outcome and risk factors for progression and survival of FMF patients. We conducted a single‐center retrospective study and reviewed 53 patients diagnosed with FMF between 1990 to 2019 in a referral center at Ankara University, Turkey. Regarding to stage at diagnosis, 24 patients (45.3%) had advanced‐stage disease (≥IIB). Follicular papules was observed in 66% and alopecia in 49.1% of the cases. Forty‐three patients (81.1%) suffered from pruritus. The majority of the patients (92.4%) had at least one systemic therapy. Complete remission was achieved in 24.5% of the patients. The median time of overall survival (OS) was 50 months (range 9‐324 months) and 5‐year and 10‐year OS was 83% and 69%, respectively. Twenty‐eight (52.3%) patients progressed to more advanced stages and seven (13.2%) patients died due to MF during the follow‐up period. FMF is associated with a progressive course and in most patients, skin‐directed therapies were found to be inefficient to control the disease and multiple systemic therapeutic agents were required to control the disease.

Updated review on prognostic factors in mycosis fungoides and new skin lymphoma trials

“…Stage IIA showed higher LDH levels (43.5%) compared to stage IA and IB (6.7% and 8.9%, respectively) 4 . In folliculotropic MF patients, elevated LDH levels were associated with 5.53 times increased mortality compared to FMF patients with normal LDH levels 25 …”

“…FMF can present like classic MF with patches, plaques, tumors, and erythroderma. However, FMF can also manifest with acneiform lesions, grouped follicular lesions, and plaque lesions involving the head and neck and extremities 25,43 . In one study, head and neck involvement was observed in 75% of FMF patients with advanced stage and was associated with poor prognosis 25 .…”

“…However, FMF can also manifest with acneiform lesions, grouped follicular lesions, and plaque lesions involving the head and neck and extremities 25,43 . In one study, head and neck involvement was observed in 75% of FMF patients with advanced stage and was associated with poor prognosis 25 . In FMF lesions located on the head and neck, pronounced plaques may develop into tumors and cause alopecia 44 .…”

“…4 In folliculotropic MF patients, elevated LDH levels were associated with 5.53 times increased mortality compared to FMF patients with normal LDH levels. 25…”

“…25,43 In one study, head and neck involvement was observed in 75% of FMF patients with advanced stage and was associated with poor prognosis. 25 In FMF lesions located on the head and neck, pronounced plaques may develop into tumors and cause alopecia. 44 Previous studies have reported an alopecia rate between 21-81% among FMF patients and head and neck involvement was related to tumoral lesions.…”