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Spiky/keratosis‐pilaris‐like early follicular mycosis fungoides: A clinicopathologic study of 20 cases with extended follow‐up
Author(s) -
Tomasini Carlo,
Michelerio Andrea,
Quaglino Pietro
Publication year - 2021
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.14002
Subject(s) - mycosis fungoides , medicine , dermatology , lymphoma , lesion , hyperkeratosis , retrospective cohort study , stage (stratigraphy) , peripheral t cell lymphoma , keratosis , cutaneous lymphoma , follicular phase , pathology , paleontology , immune system , t cell , immunology , biology
Backgrounds Follicular mycosis fungoides (FMFs) 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 FMF between 2008 and 2017 was conducted. Results Twelve males and eight 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 of 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 two patients. Disease stage at diagnosis was IA in two 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 one patient progressed to tumor stage (IIB) five years after the first diagnosis. The mean follow‐up was seven 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 identifying prognostic factors.