title: Spiky follicular mycosis fungoides: a clinicopathologic study of 8 cases.
abstract: BACKGROUND
  The early stages of follicular mycosis fungoides (FMF) have not been described previously in the literature.
  
  
  OBJECTIVE
  Our goal was to better categorize the clinicopathologic features of early stages of FMF.
  
  
  METHODS
  The clinical notes of patients with a diagnosis of FMF seen during the previous 5 years were reviewed to identify any cases that at presentation had only hyperkeratotic follicular lesions.
  
  
  RESULTS
  Eight patients (five male, three female) with a mean age of 55.4 years were enrolled. Noteworthy, FMF was not a clinical consideration in any of these patients initially. Patients presented with disseminated, slightly erythematous, hyperkeratotic, spiky follicular papules which, histopathologically, showed hyperkeratotic columns protruding from follicular plugging in concert with selective infiltration of the infundibular epithelium by atypical, mostly CD4+, lymphocytes. T-cell clonality was demonstrated in four of eight cases. The mean duration of the lesions before diagnosis was 17.1 months. The course was indolent in most of the cases (median follow up: 18 months), whilst progression to overt FMF was noted in two patients.
  
  
  CONCLUSIONS
  The number of cases is small and follow up relatively short.
  
  
  CONCLUSIONS
  Spiky FMF is a deceptive clinicopathologic presentation of FMF that has been poorly described and that can mimic numerous follicular disorders.
authors.affiliation: Anatomic Pathology, Azienda Ospedaliera Città della Salute e della Scienza, Turin, Piedmont, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Tomasini
authors.affiliation: Department of Dermatology, University Hospital, Zürich, Switzerland.
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.affiliation: Dermatologic Clinic, Department of Medical Sciences, University of Torino, Torino, Italy.
authors.forename: Mauro
authors.initials: M
authors.lastname: Novelli
authors.affiliation: Dermatologic Clinic, Department of Medical Sciences, University of Torino, Torino, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Fava
authors.affiliation: Department of Dermatology, Section of Dermatopathology, Istituto Dermopatico dell'Immacolata, Rome, Italy.
authors.forename: Giorgio
authors.initials: G
authors.lastname: Annessi
authors.affiliation: Franco Rongioletti. Dermatologic Clinic, University of Genoa, Genova, Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Rongioletti
authors.affiliation: Dermatologic Clinic, Department of Medical Sciences, University of Torino, Torino, Italy.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Fierro
authors.affiliation: Dermatologic Clinic, Department of Medical Sciences, University of Torino, Torino, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Quaglino
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology; D000368:Aged; D001706:Biopsy; D007644:Darier Disease / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D005138:Eyebrows / Q000002:abnormalities / Q000473:pathology; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D012878:Skin Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D013601:T-Lymphocytes / Q000473:pathology
keywords: Follicular mycosis fungoides; Hyperkeratotic spicules; Keratosis pilaris; Spiky