title: Follicular mucinosis: a critical reappraisal of clinicopathologic features and association with mycosis fungoides and Sézary syndrome.
abstract: BACKGROUND
  Beginning in 1957, patients have been described with localized alopecia characterized histopathologically by mucin deposition within hair follicles (follicular mucinosis [FM]). At least 2 distinct diagnostic entities have been proposed: one occurring in children and young adults without association with other diseases ("idiopathic" FM), the other occurring in elderly patients and associated with mycosis fungoides or Sézary syndrome ("lymphoma-associated" FM).
  
  
  OBJECTIVE
  To determine whether idiopathic and lymphoma-associated FM are distinct or related entities.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Department of Dermatology, University of Graz, Graz, Austria.
  
  
  METHODS
  Forty-four patients with FM were divided into 2 groups. Group 1 comprised 16 patients (mean age, 37.5 years) with no associated mycosis fungoides or Sézary syndrome; group 2 was made up of the other 28 (mean age, 52.2 years), who had clinicopathologic evidence of cutaneous T-cell lymphoma.
  
  
  RESULTS
  Mean age was lower in patients with idiopathic FM, but a considerable overlapping among the 2 groups was present. Location on the head and neck region was common in both groups, but most patients with lymphoma-associated FM had lesions also on other body sites. In fact, solitary lesions at presentation were common in patients with idiopathic FM (11 [68.8%] of 16 patients), but uncommon in those with lymphoma-associated FM (2 [7.1%] of 28 patients). Histopathologic findings did not allow clear-cut differentiation of the 2 groups. Finally, a monoclonal rearrangement of the T-cell receptor gamma gene was demonstrated by polymerase chain reaction analysis in about 50% of tested cases from each group.
  
  
  CONCLUSIONS
  Criteria previously reported to differentiate idiopathic from lymphoma-associated FM proved ineffective. In analogy to localized pagetoid reticulosis (Woringer-Kolopp disease), small-plaque parapsoriasis, and so-called solitary mycosis fungoides, idiopathic FM may represent a form of localized cutaneous T-cell lymphoma.
authors.affiliation: Department of Dermatology, University of Graz, Auenbruggerplatz 8, A-8036 Graz, Austria. lorenzo.cerroni@kfunigraz.ac.at.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cerroni
authors.forename: Regina
authors.initials: R
authors.lastname: Fink-Puches
authors.forename: Barbara
authors.initials: B
authors.lastname: Bäck
authors.forename: Helmut
authors.initials: H
authors.lastname: Kerl
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D000507:Mucinosis, Follicular / Q000150:complications / Q000235:genetics / Q000473:pathology*; D009182:Mycosis Fungoides / Q000150:complications*; D016133:Polymerase Chain Reaction; D012751:Sezary Syndrome / Q000150:complications*; D012878:Skin Neoplasms / Q000150:complications*