"Spiky/keratosis-pilaris-like early follicular mycosis fungoides: a clinicopathologic study of 20 cases with extended follow-up

Backgrounds: Follicular mycosis fungoides is a distinct form of T-cell lymphoma whose course is considered aggressive.
Methods: 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.
Results: 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.
Conclusions: 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.