title: Poikilodermatous mycosis fungoides: a study of its clinicopathological, immunophenotypic, and prognostic features.
abstract: BACKGROUND
  Poikilodermatous mycosis fungoides (MF) is a variant of MF, and its clinicopathological, immunophenotypic, molecular, and prognostic features have not previously been defined in the literature.
  
  
  OBJECTIVE
  The purpose of this study was to improve the data available for this variant of MF thus enabling clinicians to apply the appropriate treatment and follow-up.
  
  
  METHODS
  In a retrospective single center study we evaluated the clinical, histopathological, immunohistochemical, and molecular characteristics of patients with predominant (>50%) poikilodermatous lesions of MF.
  
  
  RESULTS
  In all, 49 patients were identified. The median age at diagnosis was 44 years (15-81 years). Of 49 patients, 43 (88%) had early stage disease (≤IIA) at diagnosis. No patients had stage IV disease at presentation. A frequent association was coexistence of lymphomatoid papulosis (9/49; 18%). Histopathology review showed a high number of cases with CD8(+) CD4(-) atypical lymphocytes (38%). After diagnosis most patients were treated with expectant or skin-directed therapy. Psoralen plus ultraviolet A therapy was most frequently used and had high response rates (83%). Five (10%) of 49 received systemic therapy. The mean follow-up was 11 years, 10 months (1->40 years). In all, 47 (96%) of 49 patients had stable disease and two (4%) of 49 had progressive disease. No patients died during follow-up.
  
  
  CONCLUSIONS
  As a tertiary center our patient cohort may be expected to have more advanced and aggressive disease.
  
  
  CONCLUSIONS
  Poikilodermatous MF represents a distinct clinicopathological entity from classic patch/plaque MF. It presents at a younger age and is more frequently associated with lymphomatoid papulosis. There is an increased number of cases with predominantly CD8(+) CD4(-) atypical lymphocytes. Overall there is a good response to phototherapy and the overall prognosis appears favorable.
authors.affiliation: Guy's and St Thomas' National Health Service Foundation Trust, London, United Kingdom. Electronic address: rachel.abbott@nhs.net.
authors.forename: Rachel A
authors.initials: RA
authors.lastname: Abbott
authors.affiliation: Guy's and St Thomas' National Health Service Foundation Trust, London, United Kingdom.
authors.forename: Debjani
authors.initials: D
authors.lastname: Sahni
authors.affiliation: Guy's and St Thomas' National Health Service Foundation Trust, London, United Kingdom.
authors.forename: Alistair
authors.initials: A
authors.lastname: Robson
authors.affiliation: Guy's and St Thomas' National Health Service Foundation Trust, London, United Kingdom.
authors.forename: Nita
authors.initials: N
authors.lastname: Agar
authors.affiliation: Guy's and St Thomas' National Health Service Foundation Trust, London, United Kingdom.
authors.forename: Sean
authors.initials: S
authors.lastname: Whittaker
authors.affiliation: Guy's and St Thomas' National Health Service Foundation Trust, London, United Kingdom.
authors.forename: Julia J
authors.initials: JJ
authors.lastname: Scarisbrick
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001707:Biopsy, Needle; D001768:Blister / Q000276:immunology / Q000473:pathology / Q000628:therapy; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping / Q000379:methods; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000276:immunology / Q000473:pathology* / Q000628:therapy*; D009367:Neoplasm Staging; D010789:Phototherapy / Q000379:methods*; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012737:Sex Factors; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology* / Q000628:therapy*; D016896:Treatment Outcome; D055815:Young Adult