Backgrounds Follicular mycosis fungoides is a distinct form of T-cell lymphoma whose course is considered aggressive.
a retrospective study with long-term follow-up of 20 patients diagnosed with spiky/keratosis-pilaris-like follicular mycosis fungoides between 2008 and 2017 was conducted.
12 males and 8 females were identified, with a mean age at first diagnosis of 59 years (range 42-86). Hyperkeratotic follicular papules were the sole clinical finding in 16/20 patients. A diagnostic delay between first symptom development and initial diagnosis was frequent (mean 42 months). The head/neck region was concurrently affected only in 2 patients. Disease stage at diagnosis was IA in 2 patients (10%) and IB in 18 (90%). Five patients had almost complete lesion regression, whilst there was only a slight improvement, without regression in 14. Two patients developed infiltrated papules, comedones and small cysts during follow-up. Only 1 patient progressed to tumor stage (IIB) 5 years after first diagnosis. The mean follow-up was 7 years (range: 12-180 months). None of them died of cutaneous lymphoma.
FMF presenting with only spiky/keratosis-pilaris-like lesions have an excellent prognosis at medium term follow-up. Early recognition of patients with this peculiar FMF presentation might lead to identify prognostic factors. This article is protected by copyright. All rights reserved.

This article is protected by copyright. All rights reserved.